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DIT-Things to know 2 Flashcards

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1
Q

MEN1

A 
Parathyroid adenoma
Pituitary adenoma
Pancreatic tumor
- Gastrinoma
- Insulinoma (hypoglycemia)
- Glucagonoma (hyperglycemia and diabetes)
- VIPoma (diarrhea)
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2
Q

MEN2A

A

Medullary thyroid cancer
Pheochromocytomas
Parathyroid hyperplasia

RET oncogene

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3
Q

MEN2B

A

Medullary thyroid cancer
Pheochromocytoma
Mucosal neuromas

RET oncogene

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4
Q

What might a lab detect in the urine or plasma of a patient with pheochromocytoma?

A

Plasma

  • Metanephrine
  • Normetanephrine

Urine
- Vanillylmandelic acid (VMA)

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5
Q

Most common tumor the adrenal gland

Most common tumor of the adrenal medulla (in adults)

Most common tumor of the adrenal medulla (in children)

Medical treatment for pheochromocytoma

Pheochromocytoma, medullary thyroid cancer, hyperparathyroidism

Pheochromocytoma, medullary thyroid cancer, mucosal neuromas

A
  1. Benign, non-functioning adrenal adenoma
  2. Pheochromocytoma
  3. Neuroblastoma
  4. Non-selective alpha blocker
  5. MEN2A
  6. MEN2B
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6
Q

Which cell wall inhibitor matches each of the following?

  1. Next step in tx of otitis media if amoxicillin resistant
  2. Prophylaxis against bacterial endocarditis
  3. Sufficient for the treatment of syphilis
  4. Single-dose treatment for gonorrhea
A
  1. Amoxicillin + Clavulonic avid
  2. Penicillin V, Aminopenicillins, 1st gen cepahlosporins
  3. Penicillin G
  4. Ceftriaxone
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7
Q

Equation for confidence interval

A

CI= mean +- Z (SEM)

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8
Q

Stages of grief

A 
Denial
Anger
Bargaining
Grief
Acceptance
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9
Q

In what gestational week does they thyroid begin to develop?

Embryonic origin?

A

Third week of gestation

Derived from endoderm from the floor of the primitive pharynx

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10
Q

Remnants of thyroid development

A

Thyroglossal duct

  • connection from tongue to thyroid
  • infection risk

Foreamen cecum

  • more common
  • middle of tongue
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11
Q

What is the most common site of ectopic thyroid tissue?

A

Tongue

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12
Q

Hypothyroidism will cause elevation in what labs?

A

LDL and Total cholesterol

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13
Q

Formation of thyroid hormone

A
  1. Na gradient brings Iodide (I-) into follicle
  2. Iodide is oxidized by Peroxidase to iodine (I2)
  3. Organification= Iodine binds to thyroglobulin (Tg)
  4. Thyroid hormone is formed inside they thyroglobulin by union of tyrosine and iodine
  5. Coupling or condensation of MIT and DIT –> T3 and T4
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14
Q

What converts T4 –> T3 in periphery

A

Propylthiouracil

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15
Q

Labs seen in Hypothyroidism

A

Increased TSH

Decreased T3 T4

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16
Q

Baby with impaired growth
Intellectual disability
Enlarged tongue
Distended abdomen

What could of fixed this

A

Iodine to mother during development

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17
Q

Hashimoto thyroiditis

  • type of rxn
  • infiltrate
  • genes
  • increased risk of
A

Type IV hypersensitivity
Lymphocytic infiltrate
HLA-DR5 and HLA-B5

Risk: B cell lymphoma of thyroid

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18
Q

Painful Goiter

  • infiltration
  • triggered by
  • Tx
A

Subacute (granulomatous, de Quervain) thyroiditis

Granulomatous infiltration

Viral infection

  • Coxsackievirus
  • Echovirus
  • Adenovirus
  • Measles
  • Mumps

NSAIDS
Corticosteroids

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19
Q

Fixed, hard painless goiter

  • tissue
  • infiltrate
  • may see
A

Riedel’s thyroiditis

Chronic inflammation of thyroid –> replaced by fibrous tissue

Hypothyroid
Euthyroid

Macrophages and eosinophils

Extension of fibrosis into local structures (airway)

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20
Q

Pregnancy affect on serum thyroid hormone levels

A

Pregnancy = increased estrogen

Estrogen increases thyroid binding globulin (TBG)

Binds up circulating thyroid hormone, thyroid senses T3/T4 dropping so releases more

Total T3/T4 increased
Free T3/T4 normal

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21
Q

Hyperthyroidism vs thyrotoxicosis

A

Hyperthyroidism: thyroid is making too much thyroid hormone

Thyrotoxicosis: too much thyroid hormone from any reason

  • Exogenous hormone
  • Thyroid inflammation
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22
Q

Graves disease MOA

  • Associated with
  • key features
  • Tx
A

Autoimmune

TSI binds to the TSH receptor and stimulates the thyroid gland to secrete T3 and T4

HLA-DR3 and HLA-B8

Exophthalmos
Pretibial myxedema
- Abnormal connective tissue deposit in orbit, extraocular muscle or skin on front of shins

Methimazole (preferred)
Propylthiouracil (PTU)
Beta blocker (tachycardia and anxiety)

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23
Q

Benign tumor that can present with hyperthyroidism

A

Struma ovarii teratoma

Contains functional thyroid tissue

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24
Q

Most likely diagnosis of patient with hyperthyroidism?

  1. Extremely tender thyroid gland
  2. Pretibial myxedema
  3. Pride in recent weight loss, medical professional
  4. Palpation of single thyroid nodule
  5. Palpation of multiple thyroid nodules
  6. Recent study using IV contrast (iodine)
  7. Eye changes: proptosis, edema, injection
  8. History of thyroidectomy or radioablation of thyroid
A
  1. Subacute (de Quervain) thyroiditis
  2. Graves disease
  3. Thyroid hormone abuse
  4. Toxic thyroid adenoma
  5. Multinodular goiter
  6. Jed0Basedow phenomen
  7. Graves disease
  8. Too much exogenous thyroid hormone
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25
A Tall thin male teenager has abrupt onset of dyspnea and left-sided chest pain. Percussion on the affected side reveals hyper-resonance, and breath sounds are diminished. What is most likely?
Spontaneous pneumothorax
26
Attributable risk
(A/ A+B) - (C/ C+D)
27
Ground glass appearance of cytoplasm of thyroid
Papillary thyroid cancer
28
Orphan annie eyes
Papillary thyroid cancer
29
Concentric calcification
Psammoma bodies Papillary thyroid cancer
30
Mutation of papillary thyroid cancer
RET gene mutation | BRAF
31
Uniform cuboidal cells lining follicles and invading capsule - spread - associated with
Follicular thyroid carcinoma Hematogenously RAS mutation PAX8-PPAR gamma 1 rearrangment
32
Tumor of thyroid Increased level of calcitonin Associated with Mutation
Medullay thyroid carcinoma Proliferation of parafollicular C cells RET gene mutation MEN 2A and MEN2B
33
Tumors that activate tyrosine kinase receptor
Follicular thyroid carcinoma | Medullary thyroid carcinoma
34
Rock hard thyroid with local extensions | Elderly patient
Anaplastic thyroid carcinoma Undifferentiated Rapidly fatal
35
What type of cancer in thyroid? 1) Arises from parafollicular C cells 2) RAS mutation or a PAX8-PPARy-1 rearrangment 3) Rearrangement in RET oncogene 4) Mutation in BRAF gene 5) Enlarged thyroid cells with ground-glass nuclei
1) Medullary thyroid carcinoma 2) Follicular thyroid carcinoma 3) Medullary thyroid carcinoma, papillary carcinoma 4) Papillary carcinoma 5) Papillary thyroid cancer
36
What nerve can be damaged in thyroid surgery Result?
Recurrent laryngeal n. Hoarseness
37
Number needed to harm
1/ AR AR= (A/A+B) - (C/C+D)
38
Cell types and secretion in pancreas
Alpha= Glucagon Beta= Insulin Delta= somatostatin
39
Glucagon does what
Stimulates - Gluconeogenesis - Glycogenolysis - Lipolysis (break down of fat adn TG to produce ketones) - Insulin secretion
40
GLUT-2 GLUT-4 GLUT-1
GLUT-2 (independent) - beta cells - Liver - Small intestine - Renal cells GLUT-4 (Insulin dependent) - Adipose tissue - Skeletal muscle GLUT-1 (independent) - Brain and RBCs
41
What glut transport is dependent on insulin
GLUT-4 - Adipose tissue - Skeletal muscle
42
Insulin binds to what of fat and muscle cells Does what
Tyrosine kinase receptor Causes cell to insert more GLUT-4 transporters into membrane
43
Type 1 DM - Ab against - Association
Islet cell antibody against glutamic acid decarboxylase (GAD) HLA-DR3-DQ2 HLA- DR4- DQ8
44
Hemoglobin A1C
Moiety on hemoglobin molecule as it floats around, glucose sticks to it in an non-enzymatic reaction called glycation More glucose in blood the more it sticks to hemoglobin Measure percentage of surface area covered in glucose= A1C
45
Kimmelstiel wilson nodules
Acellular nodules in glomerulus Diabetes
46
Too much glucose causes what complications
Leaky vessels Retinopathy --> hemorrhage --> blindness Nephropathy --> progressive proteinuria Vascular disease --> astherosclerosis
47
Alcohol sugar Alcohol form of glucose Glucose --> (this alcohol sugar)
Sorbitol Aldose reductase
48
Sorbital --> Fructose
Sorbitol dehydrogenase
49
Increased glucose --> sorbitol effect on tissues
Some tissues dont have srbitol dehydrogenase Trapped in cells - Schwann cells - Lens - retina - kidney Causes osmotic pressure that draws in free water causes swelling and damage to cell Motor neuropathy Sensory neuropathy Autonomic neuropathy
50
How is hemoglobin glycosylated in DM to form HgbA1C
Nonenzymatic glycation
51
Diabetes with a strong genetic predisposition
Type II DM
52
Polymyositis vs Polymyalgia rheumatica
Polymyositis - Muscle weakness - Elevated Ck and aldolase - ANA - Anti-Jo 1 Polymyalgia rheumatica - Joint pain - Normal Ck and aldolase - No muscle pain or weakness
53
Diabetic ketoacidosis - values - complications
``` Hyperglycemia > 150 Hyperkalemia - Excess H, pull H out of cell in exchange for K - Dump K in urine -Total K low ``` Arrhythmias - serum hyperkalemia, total body potassium depletion V tach Torsades Invasive fungal infections - Mucormycosis (brain abscess)
54
Extreme hyperglycemia > 800
Hyperosmolar Hyperglycemic state Type II DM
55
How do acidosis and alkalosis affect extracellular K concentrations?
Acidosis - Increases extracellular K concentration - Pushes H into cells Alkalosis - Decreases extracellular K concentration
56
What is Budd-Chiari syndrome
Occlusion of IVC or hepatic veins --> hepatic congestion
57
Which ECG leads will show evidence of ischemia in an inferior wall MI?
II, III, aVF
58
What are the findings of Brown-Sequard syndrome
Hemisection of spinal cord Ipsilateral UMN signs below lesion (lateral corticospinal tract) Ipsilateral loss of proprioception, vibration sense, and fine touch below lesion (dorsal columns) Contralateral loss or pain and temperature sensation 2-3 segments below lesion (lateral spinothalamic tract)
59
What produces hromone leptin
Adipocytes
60
Hypothalamic nuclei that regulate hunger
1. Paraventricular nucleus 2. Dorsal medial nuclei 3. Arcuate nucleus 4. Lateral hypothalamus - Stimulated --> hunger - Inhibted by leptin - Lesion --> anorexia 5. Ventromedial nuclei - Stimulated by leptin Lateral likes lunch but lesion (or leptin) lateral you get little Ventromedial nuclei --> go medially or thinner Leptin makes you thin
61
Lipodystrophy Caused by
Distortions in the structure or function of adipose tissue Buffalo hump Leptin deficiency HIV medication - Protease inhibitors
62
5 categories of criteria for the diagnosis of metabolic syndrome
``` Abdominal obesity Elevated triglycerides Low HDL Elevated BP Elevated glucose ```
63
Liver disease associated with obesity
Nonalcoholic steatohepatitis (NASH)
64
BMI to be obese
>= 30
65
Substances known for causing methemoglobinemia
Nitrates Antimalarial drugs - Chloroquine - Primaquine Dapsone Local anesthetics - Lidocaine Sulfonamides Metoclopramide
66
Parathyroid gland cells
Chief cells: PTH Oxyphil cells - packed with mitochondria - appear at pubery - increase with age
67
Parathyroid innervation
Cervical sympathetic ganglia Doesnt directly innervated parathyroid Controls blood flow to parathyroid gland
68
PTH on bone cells
Increase bone turn over Ramps up osteoclasts Osteoclasts: dont have PTH recpetors Osteoblasts: have PTH receptor - make cytokine RANKL --> activates osteoclasts
69
Subperiosteal bone reabsorption on radial aspect of medial phalanges Tapering of distal clavicles Salt and pepper appearance skull Also see?
Osteitis Fibrosis Cystica Brown tumors of long bones Primary hyperparathyroidism
70
Secondary hyperparathyroidism from renal failure values
Increased BUN and Cr | Increased Phosphate
71
Values for secondary hyperparathyroidism from Vit D deficiency
Increased PTH Decrased Ca Decreased or normal phosphate Decreased Vit D
72
Pseudohypoparathyroidism
Mutations in gene called GNAS-1 Encodes G protein at PTH receptor in kidney Increase PTH Decrease Ca AD Albright hereditary osteodystrophy - Short stature - Obesity - Undevelopment of 4th and 5th digits - Ostitis fibrosa cystica
73
WAGR complex
Wilms tumor Aniridia (no iris) Genitourinary malformation Retardation (mental and motor) WT1 deletion
74
Extrinsic coagulation pathway
Tissue factor pathway VII X
75
Intrinsci coagulation pathway
Contact activation pathway XII XI IX X
76
Deficient in factor IX
Hemophilia B
77
Deficient in Factor VIII
hemophilia A
78
Accelerating factors for coagulation pathways
Factor V | Factor VIII
79
What inhibits the accelerating factors for the coagulation pathways
Protein C | Protein S
80
RL step of coagulation pathway
Factor X
81
Common Final pathway in coagulation
Factor X --> Xa Xa + Va activate prothrombin Thrombin cleaves fibrinogen --> fibrin Fibrin + XIIIa --> mesh
82
Warfarin inhibits
``` Factor II (thrombin) Factor VII (extrinsic) Factor IX (intrinsic) Factor X Protein C Protein S ```
83
First seen with starting Warfarin
Inhibit Protein C and Protein S Transient hyeprcoagulable state
84
What cleaves Fibrinogen to fibrin
Thrombin (Factor II) Coagulase from S. Aureus
85
What is essential for coagulation cascade
Calcium
86
Factor XI
Kallikrein Activates plasminogen --> plasmin Produces Bradykinin
87
Plasmin
Breaks down fibrin mesh
88
Prothrombin time (PT)
Add tissue factor and see how long for clot to form Test - Tissue factor pathway - Final common pathway Factor VII Factor X Factor V Factor II (prothrombin) Resported with INR
89
Partial thromboplastin time (PTT)
25-40 seconds Taking plasma add material to activate contact activation pathway ``` Factor XII Factor XI Factor IX Factor VIII Factor X Factor V Factor II (prothrombin) ```
90
Hemophilia A and B - deficiency - increases
Hemophilia A - Factor VIII deficiency Hemophila B - Factor IX deficiency Increase PTT
91
Coagulation disorders vs platelet disorders cause what type of bleeding
Coagulation disorders - macrohemorrhages Platelet disorders - Microhemorrhages
92
Vit K deficiency
Cofactor for several clotting factors Factor II, VII, IX, X, C and S diSCo started in 1972 Increase PT and PTT
93
Factor V Leiden
Hypercoagulable Makes factor Va resistant to inactivation by protein C Stays active longer --> more coagulation
94
Prothrombin G20210A mutation
Hypercoagulable Predisposes to thrombosis Mutation Guanine --> Adenine at 20210
95
Antithrombin deficiency - resistant to
Hypercoagulable Unable to inactivate thrombin Resistant to heparin
96
Protein C deficiency
Hypercoagulable Unable to inactivate factor V and VIII or deficiency in protein S
97
Which glomerular disease should be suspected most in patients with each of the following findings? 1. IF: granular pattern of immune complex deposition; LM; diffuse capillary thickening 2. IF: granular pattern of immune complex deposition; LM: hypercellular 3. IF: linear pattern of immune complex deposition 4. EM: subendothelial humps and "tram track" appearance 5. Nephritis, deafness, cataracts 6. LM: crescent formation in the glomeruli 7. LM: segmetnal sclerosis and hyalinosis 8. EM spiking on the GBM due to electron-dense subepthelial deposits
1. Membranous GN Diffuse proliferative GN 2. Acute post-streptococcal GN 3. Goodpasture syndrome 4. Membranoproliferative GN 5. Alport syndrome 6. Rapidly progressive GN 7. Focal segmental glomerulosclerossi 8. Membranous GN
98
Heparin induced thrombocytopenia - What happens - symptoms - Tx
Platelet drop Heparin binds platelet factor 4 --> autoantibody-heparin PF4 complex --> activates platelets Thrombocytopenia Hypercoagulable state Stop heparin Start direct thrombin inhibitor until platelets back up Start warfarin
99
What lab test is used to monitor adequate anticoagulation in a patient taking heparin?
PTT
100
What lab test is used to monitor adequate anticoagulation in a patient taking warfarin
PT (INR)
101
Which genetic syndrome is caused by? 1. Absence of HGPRTase 2. Deficiency of aldolase B 3. Deficiency of cystathionine synthase 4. Galactose-1- phosphate uridyl transferase deficiency --> intellectual disability, hepatosplenomegaly, cataracts 5. Deficiency of tyrosinase
1. Lesch-Nyhan 2. Fructose intolerance 3. Homocystinuria 4. Galactosemia 5. Albinism
102
What gives RBC their shape Average life span RBC
Spectrin 120 days
103
Anisocytosis Poikilocytosis Polycythemia Erythrocytosis Reticulocytes
Anisocytosis: RBCs of varying sizes Poikilocytosis: RBCs of varying shapes Polycythemia: too many RBCs Erythrocytosis: too many RBCs Reticulocytes: immature RBCs
104
Basophilic stippling
Lead poisoning
105
RBC with uniform spikes all over surface
Burr cell Echinocyte Uremia Renal failure Pyruvate kinase deficiency
106
RBC with irregular spikes in size and distribution
Spur cell Acanthocyte Abetalipoproteinemia
107
Spherical RBCs
Spherocytes Hereditary sphenocytosis
108
Fragmented RBCs
Schistocytes DIC TTP, HUS
109
RBCs looks like bulls eye
Target cells "HALT" said the hunter to his target Hemoglobin C disease Asplenia Liver disease Thalassemia
110
Cell type seen in abetalipoproteinemia
Spur cell Acanthocytes RBC with irregular spikes in size and distribution
111
Crescent shaped RBC
Sickle cell Sickle cell anemia
112
RBC with basophilic remnant of a nucleus (purple spot in RBC)
Howell-Jolly body Asplenia
113
RBC with white spot of oxidized hemoglobin
Heinz body G6PD
114
RBC with little part taken out of it
Bite cell Heinz body removed G6PD
115
Tear drop RBCs
Myelofibrosis
116
Pencil or cigar shaped cells
Elliptocytes Hereditary elliptocytosis
117
RBC covered in blue dots
Ringed Sideroblast Granules of iron Disorders of heme synthesis Found in bone marrow
118
Cell type seen with? 1. Lead poisoning 2. G6PD deficiency 3. DIC 4 Abetalipoproteinemia 5. Asplenia
1. Basophilic stippling 2. Heinz body, Bite cells 3. Schistocytes 4. Spur cells (acantholysis) 5. Howell-Jolly bodies, target cells
119
Types of Hemoglobin 1. HbA 2. HbA1c 3. HbF 4. HbS 5. HbC 6. Hb Bart's 7. HbH
1. Normal (2 alpha 2 beta) 2. Poorly controlled diabetes ( 2 alpha 2 beta-glucose) 3. Fetal hemoglobin ( 2 alpha 2 gamma) 4. Sickle cell hemoglobin ( 2 alpha 2betaS glu --> val) 5. Hemoglobin C dis (2 alpha 2 betaC glu --> lys) 6. Severe alpha thalassemia (4 gamma) 7. Severe alpha thalassemia (4 beta)
120
RBC production locations
"Young Liver Synthesizes Blood" Yolk sac Liver Spleen Bone marrow
121
An 18-year-old woman presents to the emergency department with acute onset of severe abdominal pain. She says she had a similar attack 1 year earlier after taking some barbiturates. At that time she underwent an exploratory laparotomy, which revealed nothing. The patient no longer takes barbiturates but recently started an extremely low-carbohydrate and low-calorie diet. She has a temperature of 37°C (98.6°F), a respiratory rate of 16/min, and a blood pressure of 128/83 mm Hg. Her WBC count is normal. Laboratory studies reveal a sodium level of 127 mEq/L, and urinalysis shows increased porphobilinogen levels. The physician tells the patient that she has a genetic condition involving her RBCs. What congenital disorder did the physician most likely tell the patient she has? (A) Acute intermittent porphyria (B) Fanconi’s anemia (C) Hereditary spherocytosis (D) Porphyria cutanea tarda (E) Sickle cell disease
A. Acute intermittent porphyria
122
``` A 17-year-old boy presents to the emergency department with severe abdominal pain. Laboratory tests show a deficit in uroporphyrinogen synthetase and excess δ-aminolevulinate and porphobilinogen in the urine. Which of the following symptoms would most likely also be present in this patient? (A) Chest pain (B) Hypotension (C) Neuropsychiatric disturbances (D) Polyphagia (E) Stiff neck ```
C. Neuropsychiatric disturbances Acute intermittent porphyria
123
Dark red urine Psychologic disturbances Polyneuropathy Abdominal pain - deficiency - drug that precipitated Treatment
Acute intermittent porphyria Uroporphyrinogen-1 synthase (porphobilinogen deaminase) Precipitated by - Barbiturates - Seizure durgs - rifampin - metoclopramide Give Heme and Glucose - down regulates delta-ALA synthase
124
A 27-year-old man walks into the emergency department in an agitated state. He complains of severe abdominal pain and eventually becomes combative, requiring five-point restraint. His vital signs show elevated blood pressure and tachycardia. When a straight catheter is inserted, reddish urine enters the Foley bag. The urine is negative for RBCs, and a toxicity screen is negative. His doctor suspects a porphyria; laboratory tests for urine porphobilinogen are positive. Which of the following enzyme defi ciencies is responsible for this patient’s disorder? (A) Aminolevulinate dehydratase (B) Aminolevulinate synthase (C) Ferrochelatase (D) Heme oxygenase (E) Porphobilinogen deaminase (F) Uroporphyrinogen decarboxylase (G) Uroporphyrinogen III cosynthase
E. (E) Porphobilinogen deaminase aka Uroporphyrinogen-1- synthase
125
Sun sensitivity --> blistering Tea colored urine Excess hair growth Hyperpigmentation - associated with - increased lab - deficiency
Porphyria cutanea tarda Associated with hepatitis C and alcoholism Increase LFTs Uroporphyrinogen decarboxylase AD Build up uroporphyrinogen III
126
Lead poisoning affects
delta-ALA dehydratase Ferrochelatase Increased levels of protoporphyrin
127
A 3-year-old boy presents to his pediatrician with irritability, an ataxic gait, and regression of speech to single words. During the interview, the patient is constantly putting objects in his mouth. Laboratory values are signifi cant for a hemoglobin level of 8.3 g/dL. Which of the following etiologies should be suspected in this patient? (A) Acetaminophen toxicity (B) Aspirin toxicity (C) Button battery ingestion (D) Lead poisoning (E) Organophosphate absorption (F) Tricyclic antidepressant overdose
D. Lead poisoning
128
``` Abdominal pain Renal failure Mental deterioration Foot/ wrist drop Memory loss ``` Tx
Lead poisoning EDTA Succinmer Severe poisoning in child - Dimercaprol + succimer
129
Tumors that cause erythrocytosis
"Potentially Really High Hematocrit" Pheochromocytomas Renal cell carcinoma Hepatocellular carcinoma Hemangioblastoma
130
RL step of Heme synthesis
delta-ALA synthase
131
Which substance and cofactor are required for generation of GABA
Glutamate | B6
132
Plummer-Vinson syndrome
Iron deficiency anemia Esophageal webs Dysphagia +/- atrophic glossitis
133
Alpha thalassemia trait/ minor
2 abnormal alpha alleles 2 normal alpha
134
Hemoglobin H
3 abnormal alpha alleles Lots of beta
135
Hemoglobin Bart
4 abnormal alpha Hydrops fetalis Death
136
Chipmunk face Hair on end appearance on xray of skull Target cells
Beta thalassemia Major | Increase Hemoglobin F 2 alpha, 2 gamma
137
Sideroblastic anemia Labs Treatmetn
Increased serum iron Increased ferritin Tx: B6
138
Test for beta thalassemia minor
Hemoglobin electrophoresis
139
What is the RL step of beta oxidation of fatty acids
Carnitine acyltransferase I
140
Cofactors required for function of pyruvate dehydrogenase? These cofactors are also required for?
Tender Loving Care For Noone ``` TPP Lipoic acid CoA FAD NAD ``` Alpha-ketoglutarate dehydrogenase
141
Hypersegmented neutrophils Glossitis Increased homocysteine Normal methylmalonic acid
Folate deficiency
142
``` Hypersegmented neutrophila Anemia Glossitis Neurologic deficits Increased homocysteine INcreased MMA ```
B12 deficiency
143
Perncious anemia
B12 deficiency | Intrinsic factor def
144
Anemia Increased CMV Hypersegmented neutrophils Orotic acid in urine -deficiency
Orotic aciduria Deficiency of UMP synthase
145
Causes of aplastic anemia
``` Radiation Benzene Drugs - Chloramphenicol - Cancer drugs Viral infections - ParvoB19 - EBV - HIV Fanconi anemia - inherited defect of DNA repair ```
146
Megaloblastic anemia + peripheral neuropathy
B12 deficiency
147
HIV positive patient with macrocytic anemia
Zidovudine
148
What disorder is associated with each of the following? 1. HTN + hypokalemia + metabolic alkalosis 2. Fever + night sweats + WL 3. Adrenal hemorrhage due to meningococcemia 4. Blue sclerae 5. Hyperphagia + hypersexuality + hyperorality + hyperdocility 6. Nystagmus + intention tremor + scanning speech 7. Lower extremity purpura + arthralgias + renal disease
1. hyperaldosteronism 2. B symptoms of lymphoma 3. Waterhouse frridrichsen syndrome 4. Osteogenesis imperfecta 5. Kluver-Bucy syndrome (B/L amygdala) 6. Charcot triad of MS 7. Henoch-Schelein purpura
149
Cold agglutinins - what it is - nearly always - occur with
Ab against RBC that interact more strongly at low temps IgM Occur regularly in infections with EBV or mycoplasma
150
Warm agglutinins - what is it - nearly always - Seen in
Ab against RBC that react at body temp IgG ``` Seen in: - EBV, HIV - Lupus - Malignancies (CLL, non-hodgkin lymphoma) - Congenital immune abnormalities ```
151
Direct coombs
Using Ab to detect Ab already bound to RBCs
152
Indirect coombs
New blood see if Ab bind Test prior to transfusion and screen for maternal Ab to fetus blood
153
MCHC
mean corpuscular hemoglobin content
154
``` Increased MCHC Increased RDW Splenomegaly Jaundice Pigmented gallstones ``` (+) osmotic fragility test (+) Eosin-5- maleimide
Hereditary spheocytosis Defect of proteins - Ankyrin, spectrin, Band 3, Protein 4.2 Eosin-5- maleimide - reduced binding to RBCs indicated band 3 deficiency
155
Associated with G6PD deficiency
"Spleen purges Nasty Inclusion From Damaged Cells" ``` Sulfonamides Primiquine Nitrofurantoin Isoniazid Fava Beans Dapsone Chloroquine ```
156
Paroxysmal nocturnal hemoglobinuria
RBCs missing surface markers CD55 CD59 Complement attacks and lyses RBCs Ham's test
157
A 45-year-old woman arrives in the emergency department complaining of intense pain in her upper abdomen for the past 4 hours. She had a similar episode in the past, but it went away within an hour. Her history is significant for a recent flu-like infection and a prolonged feeling of fatigue and general exhaustion. Physical examination reveals that her sclerae are icteric, her palate is abnormally pigmented, and her skin has a yellow hue. Ultrasound shows radiopaque gallstones. A Coombs’ test is negative. A peripheral blood smear shows small RBCs, several of which have no central pallor. Which of the following is the most likely cause of this patient’s condition? (A) A mutation in the gene encoding ankyrin (B) A mutation in the glucose 6-phosphate dehydrogenase gene (C) Circulating antibodies targeted against erythrocytes (D) Iron deficiency anemia (E) RBC hemolysis because of a mechanical heart valve
(A) A mutation in the gene encoding ankyrin Hereditary spheocytsosi
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A 20-year-old African-American man develops anemia after being treated for a urinary tract infection. A peripheral blood smear shows RBC lysis and precipitates of hemoglobin within the RBCs. Which of the following drug classes most likely caused his hemolytic anemia? (A) Aminoglycosides (B) Fluoroquinolones (C) Macrolides (D) Sulfonamides (E) Tetracyclines
D. Sulfonamides G6PD deficiency
159
A 30-year-old, previously healthy man from Lagos, Nigeria, passes dark brown urine 2 days after starting the prophylactic antimalarial drug primaquine. On physical examination, he appears pale and is afebrile. There is no organomegaly. Laboratory studies show that his serum haptoglobin level is decreased. Which of the following is the most likely explanation of these findings? A Antibody-mediated hemolysis B Impaired DNA synthesis C Impaired globin chain synthesis D Increased susceptibility to complement-induced lysis E Mechanical fragmentation of RBCs as a result of vascular narrowing F Oxidative injury to hemoglobin G Reduced deformability of RBC membrane
F Oxidative injury to hemoglobin G6PD deficiency
160
A 16-year-old boy notes passage of dark urine. He has a history of multiple bacterial infections and venous thromboses for the past 10 years, including portal vein thrombosis in the previous year. On physical examination, his right leg is swollen and tender. CBC shows hemoglobin, 9.8 g/dL; hematocrit, 29.9%; MCV, 92 μm3; platelet count, 150,000/mm3; and WBC count, 3800/mm3 with 24% segmented neutrophils, 1% bands, 64% lymphocytes, 10% monocytes, and 1% eosinophils. He has a reticulocytosis, and his serum haptoglobin level is very low. A mutation affecting which of the following gene products is most likely to give rise to this clinical condition? A β-Globin chain B Factor V C Glucose-6-phosphate dehydrogenase D Phosphatidylinositol glycan A (PIGA) E Prothrombin G20210A F Spectrin
D Phosphatidylinositol glycan A (PIGA) Paroxysmal nocturnal hemoglobinuria PIGA gene mutation Prevents expression of certain proteins that are required as glycolipid anchor
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11. A 32-year-old woman from Hanoi, Vietnam, gives birth at 34 weeks’ gestation to a markedly hydropic stillborn male infant. Autopsy findings include hepatosplenomegaly and cardiomegaly, serous effusions in all body cavities, and generalized hydrops. No congenital anomalies are noted. There is marked extramedullary hematopoiesis in visceral organs. Which of the following hemoglobins is most likely predominant on hemoglobin electrophoresis of the fetal RBCs? A Hemoglobin A1 B Hemoglobin A2 C Hemoglobin Bart’s D Hemoglobin E E Hemoglobin F F Hemoglobin H
C Hemoglobin Bart’s alpha-thalassemia major chr 16
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9. A 3-year-old boy from Sicily has a poor appetite and is underweight for his age and height. Physical examination shows hepatosplenomegaly. The hemoglobin concentration is 6 g/dL, and the peripheral blood smear shows severely hypochromic and microcytic RBCs. The total serum iron level is normal, and the reticulocyte count is 10%. A radiograph of the skull shows maxillofacial deformities and expanded marrow spaces. Which of the following is the most likely cause of this child’s illness? A Imbalance in α-globin and β-globin chain production B Increased fragility of erythrocyte membranes C Reduced synthesis of hemoglobin F D Relative deficiency of vitamin B12 E Sequestration of iron in reticuloendothelial cells
A Imbalance in α-globin and β-globin chain production beta-thalassemia major bronze diabetes
163
12 A 17-year-old girl has had a history of fatigue and weakness for her entire life. She has not undergone puberty. On physical examination, secondary sex characteristics are not well developed. She has hepatosplenomegaly. CBC shows hemoglobin of 9.1 g/dL, hematocrit of 26.7%, MCV of 66 μm3, platelet count of 89,000/mm3, and WBC count of 3670/mm3. The appearance of the peripheral blood smear is shown in the figure. Additional laboratory findings include serum glucose of 144 mg/dL, TSH of 6.2 mU/mL, and ferritin of 679 ng/mL. A mutation in a gene encoding for which of the following is most likely to be present in this girl? A Ankyrin B β-Globin C G6PD D HFE E NADPH oxidase
B β-Globin beta-thalassemia - intermediate severity
164
10 A 10-year-old child has experienced multiple episodes of pneumonia and meningitis with septicemia since infancy. Causative organisms include Streptococcus pneumoniae and Haemophilus influenzae. On physical examination, the child has no organomegaly and no deformities. Laboratory studies show hemoglobin of 9.2 g/dL, hematocrit of 27.8%, platelet count of 372,000/mm3, and WBC count of 10,300/mm3. A hemoglobin electrophoresis shows 1% hemoglobin A2, 7% hemoglobin F, and 92% hemoglobin S. Which of the following is the most likely cause of the repeated infections in this child? ``` (RBC white white center) A Absent endothelial cell expression of adhesion molecules B Diminished hepatic synthesis of complement proteins C Impaired neutrophil production D Loss of normal splenic function E Reduced synthesis of immunoglobulins ```
D Loss of normal splenic function Sickle cell anemia
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13 A 12-year-old boy has a history of episodes of severe abdominal, chest, and back pain since early childhood. On physical examination, he is afebrile, and there is no organomegaly. Laboratory studies show hemoglobin of 11.2 g/dL, platelet count of 194,000/mm3, and WBC count of 9020/mm3. The peripheral blood smear shows occasional sickled cells, nucleated RBCs, and Howell-Jolly bodies. Hemoglobin electrophoresis shows 1% hemoglobin A2, 6% hemoglobin F, and 93% hemoglobin S. Hydroxyurea therapy is found to be beneficial in this patient. An increase in which of the following is the most likely basis for its therapeutic efficacy? ``` A Erythrocyte production B Overall globin chain synthesis C Oxygen affinity of hemoglobin D Production of hemoglobin A E Production of hemoglobin F ```
E Production of hemoglobin F Sickle cell anemia
166
19 34-year-old woman reports becoming increasingly tired for the past 5 months. On physical examination, she is afebrile and has mild splenomegaly. Laboratory studies show a hemoglobin concentration of 10.7 g/dL and hematocrit of 32.3%. The peripheral blood smear shows spherocytes and rare nucleated RBCs. Direct and indirect Coombs test results are positive at 37° C, although not at 4° C. Which of the following underlying diseases is most likely to be diagnosed in this patient? A Escherichia coli septicemia B Hereditary spherocytosis C Infectious mononucleosis D Mycoplasma pneumoniae infection E Systemic lupus erythematosus
E Systemic lupus erythematosus Warm autoimmune hemolytic anemia secondary to SLE
167
20 A 22-year-old woman has experienced malaise and a sore throat for 2 weeks. Her fingers turn white on exposure to cold. On physical examination, she has a temperature of 37.8° C, and the pharynx is erythematous. Laboratory findings include a positive monospot (heterophile antibody) test result. Direct and indirect Coombs test results are positive at 4° C, although not at 37° C. Which of the following molecules bound on the surfaces of the RBCs most likely accounts for these findings? A α2-Macroglobulin B Complement C3b C Fibronectin D Histamine E IgE
B Complement C3b Cold agglutinin disease
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38 A 28-year-old, previously healthy man has noted increasing fatigue for the past 6 months and formation of bruises after minimal trauma. Over the past 2 days, he has developed a cough. On physical examination, his temperature is 38.9° C, and he has diffuse rales in both lungs. He has no hepatosplenomegaly and no lymphadenopathy. Laboratory findings include a sputum culture positive for Streptococcus pneumoniae, hemoglobin of 7.2 g/dL, hematocrit of 21.7%, platelet count of 23,400/mm3, WBC count of 1310/mm3, prothrombin time of 13 seconds, partial thromboplastin time of 28 seconds, and total bilirubin of 1 mg/dL. The ANA test result is negative. What is the most likely explanation of these findings? A Hematopoietic stem cell defect B Hemolysis of antibody-coated cells C Increased susceptibility to lysis by complement D Metastatic adenocarcinoma to bone marrow E Secondary hypersplenism
A Hematopoietic stem cell defect Aplastic anemia
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47 A 37-year-old woman has noted an excessively heavy menstrual flow each of the past 6 months. She also has noticed increasing numbers of pinpoint hemorrhages on her lower extremities in the past month. Physical examination shows no organomegaly or lymphadenopathy. CBC shows hemoglobin of 14.2 g/dL, hematocrit of 42.5%, MCV of 91 μm3, platelet count of 15,000/mm3, and WBC count of 6950/mm3. On admission to the hospital, she has melena and after a transfusion of platelets, her platelet count does not increase. Which of the following describes the most likely basis for her bleeding tendency? A Abnormal production of platelets by megakaryocytes B Defective platelet-endothelial interactions C Destruction of antibody-coated platelets by the spleen D Excessive loss of platelets in menstrual blood E Suppression of pluripotent stem cell division
C Destruction of antibody-coated platelets by the spleen Bleeding due to low platelet count Idiopathic chronic immune thrombocytopenia purpura (ITP)
170
48 A 9-year-old boy has developed prominent bruises on his extremities over the past week. On physical examination, he has ecchymoses and petechiae on his arms and legs. Laboratory studies show hemoglobin, 13.8 g/dL; hematocrit, 41.9%; MCV, 93 μm3; platelet count, 11,300/mm3; and WBC count, 7720/mm3. He had respiratory syncytial virus pneumonia 3 weeks ago. His condition improves with corticosteroid therapy. Which of the following abnormalities is most likely to cause his hemorrhagic diathesis? A Antiplatelet antibodies B Bone marrow aplasia C Glycoprotein IIb/IIIa dysfunction D Vitamin C deficiency E Von Willebrand factor metalloproteinase deficiency
A Antiplatelet antibodies Acute immune thrombocytopenic purpura (ITP) and chronic ITP
171
49 A 21-year-old woman known to have a protein C deficiency develops recurrent pulmonary thromboembolism and is placed on anticoagulant therapy. Two weeks after initiation of this therapy, she has a sudden change in mental status and experiences difficulty speaking and swallowing. A cerebral angiogram shows a left middle cerebral artery occlusion. Laboratory studies show hemoglobin of 13 g/dL, platelet count of 65,400/mm3, WBC count of 5924/mm3, prothrombin time of 12 seconds, and partial thromboplastin time of 51 seconds. The anticoagulant therapy is discontinued. Which of the following pharmacologic agents used as an anticoagulant in this patient is most likely to have caused these findings? A Acetylsalicylic acid (aspirin) B Heparin C Tissue plasminogen activator D Urokinase E Warfarin
B Heparin Heparin-induced thrombocytopenia
172
45 A 23-year-old woman in her 25th week of pregnancy has felt no fetal movement for the past 3 days. Three weeks later, she still has not given birth and suddenly develops dyspnea with cyanosis. On physical examination, her temperature is 37° C, pulse is 106/min, respirations are 23/min, and blood pressure is 80/40 mm Hg. She has large ecchymoses over the skin of her entire body. A stool sample is positive for occult blood. Laboratory studies show an elevated prothrombin time and partial thromboplastin time. The platelet count is decreased, plasma fibrinogen is markedly decreased, and fibrin split products are detected. A blood culture is negative. Which of the following is the most likely cause of her bleeding diathesis? A Consumption of coagulation factors B Defects in platelet aggregation C Increased vascular fragility D Reduced production of platelets E Toxic injury to the endothelium
A Consumption of coagulation factors Thrombocytopenia Increased PT and PTT Fibrin split products Low fibrinogen Disseminated intravascular coagulation (DIC)
173
50 A 56-year-old woman suffers the sudden onset of headache and photophobia, and her condition worsens for the next 2 days. On physical examination, she has a temperature of 38° C and is disoriented. CBC shows hemoglobin of 11.2 g/dL, hematocrit of 33.7%, MCV of 94 μm3, platelet count of 32,000/ mm3, and WBC count of 9900/mm3. The peripheral blood smear shows schistocytes. The serum urea nitrogen level is 38 mg/dL, and the creatinine level is 3.9 mg/dL. Which of the following is the most likely diagnosis? A Autoimmune hemolytic anemia B β-Thalassemia major C Disseminated intravascular coagulation D Idiopathic thrombocytopenic purpura E Paroxysmal nocturnal hemoglobinuria F Thrombotic thrombocytopenic purpura
F Thrombotic thrombocytopenic purpura | TTP
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54 A clinical study is performed involving adult patients diagnosed with microangiopathic hemolytic anemia. A subgroup of patients who had fever or diarrhea preceding the initial diagnosis of anemia were excluded. The patients had schistocytes present on peripheral blood smears. Some of these patients were found to have a deficiency of a metalloproteinase known as ADAMTS13. Which of the following conditions were the patients with this deficiency most likely to have? A Disseminated intravascular coagulation (DIC) B Hemolytic-uremic syndrome (HUS) C Heparin-induced thrombocytopenia (HIT) D Idiopathic thrombocytopenic purpura (ITP) E Thrombotic thrombocytopenic purpura (TTP)
E Thrombotic thrombocytopenic purpura (TTP)
175
55 A 45-year-old woman has had episodes of blurred vision and headaches for the past 6 months. She has had worsening confusion with paresthesias over the past 3 days. On physical examination, she has a temperature of 39.6° C, pulse of 100/min, respiratory rate of 20/min, and blood pressure of 80/50 mm Hg. Petechial hemorrhages are noted over her trunk and extremities. Laboratory findings include hemoglobin, 10.9 g/dL; hematocrit, 34%; MCV, 96 μm3; platelet count, 28,000/mm3; and WBC count, 8500/mm3. Fragmented RBCs are noted on her peripheral blood smear. Blood urea nitrogen is 40 mg/dL, and serum creatinine is 3.1 mg/dL. Which of the following is the most likely underlying cause for her findings? A Circulating toxin that injures capillary endothelium B Decreased factor VIII activity C Defective ADP-induced platelet aggregation D Formation of autoantibodies to platelet glycoproteins IIb/IIIa and Ib-IX E Inappropriate release of thromboplastic substances into blood F Presence of antibodies against ADAMTS13 metalloproteinase
F Presence of antibodies against ADAMTS13 ``` Neurlogic abnromalities Fever Thrombocytopenia microangiopathic hemolytic anemia Renal failure ``` Thrombotic thrombocytopenic purpura (TTP) deficiency of von Willebrand factor (vWF) metalloproteinase (ADMTS13)
176
52A 23-year-old woman has a history of easy bruising. Physical examination shows multiple bruises ranging in color from red to blue to purple on her arms and legs. There is no organomegaly, and no deformities are noted. Laboratory studies show hemoglobin, 9.5 g/dL; hematocrit, 28.2%; platelet count, 229,300/mm3; WBC count, 7185/mm3; prothrombin time, 12 seconds; and partial thromboplastin time, 38 seconds. A 1:1 dilution of the patient’s plasma with normal pooled plasma corrects the partial thromboplastin time. Ristocetin-dependent platelet aggregation in patient plasma is markedly reduced. Factor VIII activity is 30% (reference range 50% to 150%). Which of the following is the most likely potential consequence of this disease? A Bone marrow failure from aplasia B Excessive bleeding after oral surgery C Increasing difficulty with joint mobility D Myeloproliferative disorder E Recurrent deep venous thrombosis
B Excessive bleeding after oral surgery Normal platelet count Prolonged bleeding time Von Willebrand disease
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58 A 15-year-old girl has a history of easy bruising and hemorrhages. Since menarche at the age of 13 years, she has had menometrorrhagia. On physical examination, she displays joint deformity and has decreased mobility of the ankles, knees, and wrists. Laboratory studies show hemoglobin, 11.8 g/dL; hematocrit, 35.1%; platelet count, 267,000/mm3; WBC count, 5960/mm3; prothrombin time, 13 seconds; and partial thromboplastin time, 60 seconds. A 1:1 dilution of the patient’s plasma with normal pooled plasma corrects the partial thromboplastin time. Which of the following is the most likely diagnosis? A Antiphospholipid syndrome B Hemophilia B C Idiopathic thrombocytopenic purpura D Thrombotic thrombocytopenic purpura E Von Willebrand disease
B Hemophilia B PTT corrected by nromal pooled plasma Factor IX deficiency
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56 A 12-year-old boy has had worsening problems with joint mobility involving his arms and legs, particularly his knees and ankles, for the past 6 years. He has been receiving therapy for this condition. His grandfather had a similar condition and died at age 25 years. On physical examination, he has no visible petechiae or areas of purpura. Laboratory studies show that prothrombin time is 12 seconds, and partial thromboplastin time is 52 seconds. After addition of an equivalent aliquot of normal plasma, the partial thromboplastin time is 30 seconds. Hemoglobin is 12.9 g/dL, platelet count is 238,500/mm3, and WBC count is 6620/mm3. His platelet function studies are normal. What is the most likely inheritance pattern for his condition? A Autosomal dominant B Autosomal recessive C Confined placental mosaicism D Germline mutation E X-linked recessive
E X-linked recessive Hemophilia A monitored with Factor VIII
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59 A 16-year-old girl has had frequent nosebleeds since childhood. Her gums bleed easily, even with routine tooth brushing. She has experienced menorrhagia since menarche at age 13 years. On physical examination, there are no abnormal findings. Laboratory studies show hemoglobin, 14.1 g/dL; hematocrit, 42.5%; MCV, 90 μm3; platelet count, 277,400/mm3; and WBC count, 5920/mm3. Her platelets fail to aggregate in response to ADP, collagen, epinephrine, and thrombin. The ristocetin agglutination test result is normal. There is a deficiency of glycoprotein IIb/IIIa. Prothrombin time is 12 seconds, and partial thromboplastin time is 28 seconds. What is the most likely diagnosis? A Disseminated intravascular coagulation B Glanzmann thrombasthenia C Immune thrombocytopenic purpura D Vitamin C deficiency E Von Willebrand disease
B Glanzmann thrombasthenia AR
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An 8-year-old boy is brought to the pediatrician after his parents noticed very dark urine in the toilet earlier that morning. Initial laboratory studies show intravascular hemolysis; further testing shows that his RBCs are susceptible to complement-mediated lysis. This patient most likely has which of the following conditions? (A) Autoimmune hemolytic anemia (B) Common bile duct stricture (C) Hereditary spherocytosis (D) Paroxysmal nocturnal hemoglobinuria (E) Thrombotic thrombocytopenic purpura
(D) Paroxysmal nocturnal hemoglobinuria
181
6. Low-molecular-weight heparins (LMWH) are distinct from unfractionated heparin in several ways. Which of the following is the primary target of LMWH? (A) Antithrombin III (B) Factor IIa (C) Factor VII (D) Factor Xa (E) Factors II, IX, and X
(D) Factor Xa LMWH act on Xa
182
10. A 7-year-old African-American boy is brought to see his pediatrician. His father says he has noticed that the boy has been complaining of right knee pain for the past week. On physical exam, multiple ecchymoses are noted on both upper and lower extremities. His father claims that the boy has always bruised easily, and he has recently learned how to ride a bicycle. Which of the following elements of the coagulation cascade is most likely to be missing in this child? (A) Antithrombin III (B) Factor VII (C) Factor VIII (D) Factor IX (E) Protein C
(C) Factor VIII Hemophilia A X linked
183
11. A 16-year-old boy is brought to the emergency department because of the acute onset of fever, chills, and a productive cough. X-ray of the chest shows an infi ltrate restricted to the left lower lobe. Samples taken of the sputum show α-hemolytic gram-positive cocci in pairs. The patient says that he has had similar infections over the past year. A peripheral blood smear is done, and results show several sickle-shaped RBCs. Which of the following explains why this patient is susceptible to this particular type of infection? (A) Bone marrow infi ltration resulting in neutropenia and compromised immune function (B) Large vessel occlusions in the cerebral vasculature resulting in neurologic events and aspiration pneumonia (C) Microvascular infarcts resulting in pulmonary failure (D) Microvascular infarcts resulting in splenic dysfunction (E) Vaso-occlusion in the renal medulla
(D) Microvascular infarcts resulting in splenic dysfunction Sickle cell disease
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14.A child is brought to the pediatrician because her parents are concerned about lead poisoning since their house is known to contain leadbased paint. A complete blood cell count reveals anemia. Lead poisoning causes anemia because it does which of the following? (A) Disrupts heme synthesis by causing decreased iron absorption from the gut (B) Disrupts heme synthesis by increasing the activity of aminolevulinate dehydratase (C) Disrupts heme synthesis by inhibiting ferrochelatase (D) Disrupts hemoglobin function by binding to hemoglobin with high affi nity, preventing oxygen binding (E) Disrupts RBC DNA synthesis, causing megaloblastic changes in RBCs
(C) Disrupts heme synthesis by inhibiting ferrochelatase ``` Lead inhibits (ALA) dehydratase Ferrochelatase ```
185
27 A 3-year-old girl was in her usual state of good health 1 month ago when she developed an acute viral upper respiratory infection. She now presents to the emergency department with nonblanching purple skin lesions. The rest of her physical examination is unremarkable. The complete blood count demonstrates a low platelet count, while the peripheral blood smear is notable only for large platelets. Which of the following laboratory fi ndings would most likely be present in this patient? (A) Antiplatelet antibodies (B) Decreased megakaryocytes on bone marrow biopsy (C) IgA defi ciency (D) Increased fi brin split products (E) Vitamin K defi ciency
(A) Antiplatelet antibodies Idiopathic thrombocytopenic purpura (ITP)
186
36 Hydrops fetalis occurs in the setting of a certain type of thalassemia. What is the underlying mechanism leading to this event? (A) Excess α-globin chains binding tighter to oxygen (B) Excess α-globin chains binding weaker to oxygen (C) Excess β-globin chains binding tighter to oxygen (D) Excess β-globin chains binding weaker to oxygen (E) Excess gamma-globin chains binding tighter to oxygen (F) Excess gamma-globin chains binding weaker to oxygen
(E) Excess gamma-globin chains binding tighter to oxygen alpha thalassemia
187
40 A 28-year-old woman comes to the physician concerned about an excessive amount of bleeding from her gums when she brushes her teeth. Her laboratory results show an increased partial thromboplastin time and an increased bleeding time, but are otherwise unremarkable. Which of the following treatments will most likely alleviate this patient’s symptoms? (A) Cryoprecipitate (B) Factor VIII concentrate (C) Fresh frozen plasma (D) Low-molecular-weight heparin (E) Protamine sulfate
(A) Cryoprecipitate Von Willebrand disease
188
42 A 62-year-old woman presents to the clinic complaining of frequent bleeding while brushing her teeth and easy bruising. She reports she recently had pneumonia and was treated with a broad-spectrum antibiotic. Laboratory tests show: Prothrombin time: 18 seconds Partial thromboplastin time: 37 seconds Platelet count: 231,000/mm³ Hematocrit: 37% WBC count: 4800/mm³ The cofactor that is defi cient in this patient is needed for the carboxylation of glutamate residues of which of the following? (A) Factors II, VII, VIII, and X (B) Factors VII, VIII, IX, and XII (C) Proteins C and S and factors IX, X, XI, and XII (D) Proteins C and S and factors XII, IX, and X (E) Proteins C and S, prothrombin, and factors VII, IX, and X
(E) Proteins C and S, prothrombin, and factors VII, IX, and X Prolong pT Deficiency of factor in extrinsic pathways
189
43. A 20-year-old African-American man develops anemia after being treated for a urinary tract infection. A peripheral blood smear shows RBC lysis and precipitates of hemoglobin within the RBCs. Which of the following drug classes most likely caused his hemolytic anemia? (A) Aminoglycosides (B) Fluoroquinolones (C) Macrolides (D) Sulfonamides (E) Tetracyclines
(D) Sulfonamides | G6PD deficiency
190
44 Several drugs are used to prevent myocardial infarction in patients with acute coronary syndrome. One class of drugs binds to the glycoprotein receptor IIb/IIIa on activated platelets, thereby interfering with platelet aggregation. This prevents renewed formation of clots that could block the lumen of the cardiac vessels. Which of the following is an example of this class of drug? (A) Abciximab (B) Clopidogrel (C) Leuprolide (D) Selegiline (E) Ticlopidine
(A) Abciximab
191
47 A 70-year-old man comes to his physician for a routine physical examination. Although he is asymptomatic, a blood test shows an abnormal level of immunoglobulin. After further testing, he is diagnosed with monoclonal gammopathy of undetermined signifi cance. Which of the following is the current treatment for monoclonal gammopathy of undetermined signifi - cance? (A) Alendronate (B) Anticoagulation (C) High-dose steroids (D) No treatment (E) Vinca alkaloids
(D) No treatment Monoclonal gammopathy of undetermined significant (MGUS)
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48 An 8-year-old boy has a chronic history of severe hemolytic anemia, hepatosplenomegaly, and maxillary overgrowth. He has received blood transfusions since early infancy but has not received a transfusion in over 4 months. A hemoglobin electrophoresis shows marked elevation of HbF, increased HbA2, and absence of HbA1. Which of the following diagnoses is most consistent with this patient’s electrophoresis? (A) α-Thalassemia minor (B) β-Thalassemia major (C) β-Thalassemia minor (D) Glucose 6-phosphate dehydrogenase defi - ciency (E) HbH disease (F) RBCs containing hemoglobin Barts
(B) β-Thalassemia major Thalassemias
193
Sickle cell disease - mutation - xray - tx
HbS mutation Point mutation on beta globulin Glu --> Val Hair on end appearance on Xray skull -- bone marrow hyperplasia Hydroxyurea -increases production of HbF
194
Order of bands on electrophoresis
``` Left to right C S F A ```
195
Ham's test Osmotic fragility test
Paroxysmal nocturnla hemoglobinuria Hereditary spherocytosis
196
Women develops muscle cramps and darkening of urine after exercising. Diagnosis?
McArdle disease
197
Von Willebrand factor (vWF) - fxn - works with - defieicny
Complexes with and stabilizes factor VIII Platelet adhesion to vessel wall and other platelets Deficiency increase PTT Increase bleeding time
198
Life span platelets
8-10 days
199
Platelet Adhesion steps
Endothelial damage Exposure underlying collagen Damaged cells release contents vWF binds exposed collagen and bind glycoprotein receptor GpIb on surface of platelet
200
Platelet Activation Platelet Aggregation
Activation - platelets grow pseudopods help bind in injury site - secrete substances Aggregation - Activated platelet secretes ADP (adenosine diphosphate) - ADP binds to receptor on surface of platelet - Triggers expression of glycoprotein IIB/IIIA to come to surface of platelet - binds fibrinogen, forms series of crosslinks between platelets
201
Branching rods on oral infection
Actinomyces israelii
202
Eczema Recurrent infections Thrombocytopenia
Wiskott-aldrich syndrome
203
Hemosiderinuria | Thrombosis
Paroxysmal nocturnal hemoglobinuria
204
Deficiency of metalloprotease ADAMTS13
Thrombotic thrombocytopenic purpura (TTP)
205
Thrombotic thrombocytopenic purpura (TTP)
Deficiency of metalloprotease ADAMTS13 ADAMTS13 cleaves vWF into active units Unregulated platelet aggregation --> thrombosis Consumption of paltelets --> thrombocytopenia Microangiopathic hemolytic anemia
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Platelet disorder presentation
Bleeding from mucous membranes Epistaxis (nose bleeds) Petechiae Purpura Increased bleeding time Normal PT and PTT
207
Immune thrombocytopenia (ITP) - labs - tx
Autoantibodies bind glycoprotein IIb/IIIa on surface platelets Decreased platelets Increased megakaryocytes (trying to make more platelets) Steroids IVIG Splenectomy
208
Thrombotic thromocytopenic purpura symptoms
``` "HUNT For The TPP" Hemolysis Uremia Neurological symptoms Thrombocytopenia Fever TTP ```
209
Hemolytic uremic syndrome associated with features
E.coli 0157:H7 Children Hemolysis Renal insufficiency Thromobytopenia
210
Bernard-Soulier syndrome
Defect of glycoprotein Ib Platelet unable to bind collagen Moderate thrombocytopenia Platelet number only slightly low Increased bleeding time
211
Glanzmann thrombasthenia
Defect in glycoprotein IIb/IIIa Abnormal platelet aggregation Normal platelet count Increased bleeding time
212
Increased PTT | Increased bleeding time
Von Willebrand disease
213
Ristocetin cofactor assay
Causes platelets to agglutinate if vWF is present
214
Von Willebrand disease | tx
DDAVP - desmopressin (ADH analog) ADH increase release of vWF from storage sites in endothelial cells
215
``` Decreased platelets Increased bleeding time Increased PT and PTT Decreased fibrinogen Increase fibrin split products (d-dimer) ```
Disseminated intravascular coagulation (DIC)
216
Which matches? 1. Anti-gp IIb/IIIa antibodies 2. ADAMTS-13 deficiency 3. gp Ib defect 4. gp IIb/ IIIa defect 5. Von Willebrand factor defect 6. Widespread activation of platelets and coagulation cascade
1. ITP 2. TTP-HUS 3. Bernard-Soulier syndrome 4. Glanzmann thromasthenia 5. von Willebrand disease 6. DIC
217
Antiplatelet antibodies
Immune thrombocytopenia
218
Large cells with bi-lobed nucleus Prominent nucleoli with clearing around nucleoi Clearing around cell
Reed Sternberg cells Hodgkin lymphoma
219
``` 48 A 35-year-old woman presents to her primary care physician with a fever of 38.3º C (101º F), night sweats, and fatigue. The patient says that she has lost about 6.7 kg (15 lb) over the past year. A CT scan demonstrates mediastinal lymphadenopathy. Biopsy of the nodes shows a small number of large cells with “owl-eye” nucleoli, multiple nuclei, and an abundance of pale cytoplasm on a background of many reactive lymphocytes, macrophages, and granulocytes. Which of the following drugs could be used to treat this condition? (A) Azathioprine (B) Cisplatin (C) Doxorubicin (D) Paclitaxel (E) β-Interferon ```
C. Doxorubicin Hodgkin lymphoma
220
4. A 34-year-old man presents to his primary care physician with night sweats, a fever of 38º C (100.2º F), and weight loss of 5 kg (12 lb) over the last 3 months. A CT scan demonstrates mediastinal lymphadenopathy, and results of a biopsy of the node are shown in the image. Which of the following drugs is part of the multidrug regimen that would be used to manage this patient’s disease? ``` (A) Cyclosporine (B) Hydroxyurea (C) Imatinib (D) Isoniazid (E) Vinblastine ```
E.(E) Vinblastine ``` ABVD Adriamycin Bleomycin Vinblastine Decarbazine ```
221
Hodgkin lymphoma in younger male
Lymphocyte-prdominant type
222
Few reed sterberg to lymphocytes Bands of collagen, sclerosis and fibrosis Women
Nodular sclerosing | Hodgkin lymphoma
223
t(14;18)
Diffuse large B cell lymphoma -Elderly Follicular lymphoma
224
t (8;14)
Burkitt lymphoma c-MYC Starry sky appearance
225
Type of burkitt lymphoma
Endemic - Africa - involves mandible Sporadic - pelvis or abdomen Immunodeficiency assoc - HIV
226
t(11;14)
Mantle cell lymphoma Disruption of regulation of cyclin D
227
Non-hodgkin lymphoma associated with sjogren, hashimoto and h. pylori
Marginal cell MALToma
228
Most common lymphoma in U.S
Diffuse large B cell lymphoma
229
Owl eye nuclei
Reed-sternberg cels | Hodgkin lymphoma
230
Lymphoma associated with EBV
Burkitt lymphoma | Hodgkin lymphoma
231
Over 20% blasts in bone marrow
AML or ALL
232
``` Child Bone pain terminal deoxynucleotidy transferase (TdT) 20% blasts t(9;22) ```
ALL
233
Auer rods 20% blasts Adult
AML
234
Elderly adult CD19 CD 20 Smudge cells
CLL
235
``` t(9;22) Fatigue Abdominal pain Splenomegaly Middle aged ```
CML
236
t(9;22) - is what - creates - associated with - tx
Philadelpha chromosome Mutation bcr-abl Oncogene Activated tyrosine kinase always on CML: always present ALL: Sometimes present AML: rarely present Tx: imatinib - inhibits bcr-abl tyrosine kinase
237
t(15;17) tx
M3 AML Treat w/ all-trans retinoic acid
238
Acute luekemia positive for peroxidase
AML
239
Bence Jones proteins
Multiple myeloma
240
Rouleaux formation
RBC stacked like coins MM
241
33 32-year-old woman presents to the emergency department with a temperature of 38.7º C (101.7º F), petechiae covering her abdomen, and mental status changes. On questioning, her husband states that “she had been fi ne 3 weeks ago but got sick real fast.” Physical examination shows severe weakness, petechiae and ecchymoses on the abdomen and back, and pale conjunctiva. A laboratory study shows severe anemia and thrombocytopenia with leukocytosis. The peripheral blood smear demonstrates the presence of abundant myeloblasts. Which of the following chromosomal translocations is most likely involved in this disorder? (A) t(8:14) c-myc activation (B) t(9:22), bcr-abl (C) t(11:14), BCL1/PRAD1 (D) t(11:22), EWS gene (E) t(15:17), PML-RAR-α
(E) t(15:17), PML-RAR-α AML
242
15 A 70-year-old man has experienced increasing fatigue for the past 6 months. On physical examination, he has nontender axillary and cervical lymphadenopathy, but there is no hepatosplenomegaly. The CBC shows hemoglobin, 9.5 g/dL; hematocrit, 28%; MCV, 90 μm3; platelet count, 120,000/mm3; and WBC count, 42,000/mm3. His peripheral blood smear shows a monotonous population of small, round, maturelooking lymphocytes. Flow cytometry shows these cells to be CD19+, CD5+, and deoxynucleoti dyl transferase negative (TdT−). Cytogenetic and molecular analysis of the abnormal cells in his blood are most likely to reveal which of the following alterations? A Clonal rearrangement of immunoglobulin genes B Clonal rearrangement of T-cell receptor genes C t(8;14) leading to c-MYC overexpression D t(9;22) leading to BCR-ABL rearrangement E t(14;18) leading to BCL2 overexpression
A Clonal rearrangement of immunoglobulin genes CLL
243
17 A 4-year-old boy has appeared listless during the past week. He exhibits irritability when his arms or legs are touched. In the past 2 days, large ecchymoses have appeared on the right thigh and left shoulder. CBC shows hemoglobin, 9.3 g/dL; hematocrit, 28.7%; MCV, 96 μm3; platelet count, 45,000/mm3; and WBC count, 13,990/mm3. Examination of the peripheral blood smear shows blasts that lack peroxidasepositive granules, but contain PAS-positive aggregates and stain positively for deoxynucleoti dyl transferase negative (TdT−). Flow cytometry shows the phenotype of blasts to be CD19+, CD3−, and sIg−. Which of the following is the most likely diagnosis? A Acute lymphoblastic leukemia B Acute myelogenous leukemia C Chronic lymphocytic leukemia D Chronic myelogenous leukemia E Idiopathic thrombocytopenic purpura
A Acute lymphoblastic leukemia ALL
244
8 A 36-year-old woman has a cough and fever for 1 week. On physical examination, her temperature is 38.3° C. She has diffuse crackles in all lung fields. A chest radiograph shows bilateral extensive infiltrates. CBC shows hemoglobin, 13.9 g/ dL; hematocrit, 42%; MCV, 89 μm3; platelet count, 210,000/ mm3; and WBC count, 56,000/mm3 with 63% segmented neutrophils, 16% bands, 7% metamyelocytes, 3% myelocytes, 1% blasts, 8% lymphocytes, and 2% monocytes. A bone marrow biopsy is obtained and shows normal maturation of myeloid cells. Which of the following is the most likely diagnosis? A Chronic myelogenous leukemia B Hairy cell leukemia C Hodgkin lymphoma, lymphocyte depletion type D Leukemoid reaction E Myelodysplastic syndrome
D Leukemoid reaction CML
245
14 A 14-year-old boy complains of discomfort in his chest that has worsened over the past 5 days. On physical examination, he has generalized lymphadenopathy. A chest radiograph shows clear lung fields, but there is widening of the mediastinum. A chest CT scan shows a 10-cm mass in the anterior mediastinum. A biopsy specimen of the mass is obtained and microscopically shows effacement by lymphoid cells with lobulated nuclei having delicate, finely stippled, nuclear chromatin. There is scant cytoplasm, and many mitotic figures are seen. The cells express deoxynucleoti dyl transferase negative (TdT−), CD2, and CD7 antigens. Molecular analysis reveals a point mutation in the NOTCH1 gene. The oncologist tells the parents that chemotherapy can be curative in vast majority of such cases. What is the most likely diagnosis? A Burkitt lymphoma B Follicular lymphoma C Hodgkin lymphoma, nodular sclerosing type D Lymphoblastic lymphoma E Mantle cell lymphoma F Small lymphocytic lymphoma
D Lymphoblastic lymphoma ALL
246
18 A 7-year-old boy has complained of a severe headache for the past week. On physical examination, there is tenderness on palpation of long bones, hepatosplenomegaly, and generalized lymphadenopathy. Petechial hemorrhages are present on the skin. Laboratory studies show hemoglobin, 8.8 g/dL; hematocrit, 26.5%; platelet count, 34,700/mm3; and WBC count, 14,800/mm3. A bone marrow biopsy specimen shows 100% cellularity, with almost complete replacement by a population of large cells with scant cytoplasm lacking granules, delicate nuclear chromatin, and rare nucleoli. His oncologist is confident that chemotherapy will induce a complete remission. Which of the following combinations of phenotypic and karyotypic markers is most likely to be present in marrow cells from this boy? A Early pre-B CD19+ hyperdiploidy B Early pre-B CD20+ t(9;22) C Pre-B CD5+ normal karyotype D Pre-B CD23+ 11q deletion E T cell CD2+ numerous blasts F T cell CD3+ MLL gene translocation
A Early pre-B CD19+ hyperdiploidy ALL
247
20 The figure skater Sonja Henie, who won gold medals at the 1928, 1932, and 1936 Winter Olympic Games, became progressively fatigued in her late 50s. On physical examination, she had palpable nontender axillary and inguinal lymph nodes, and the spleen tip was palpable. Laboratory studies showed hemoglobin, 10.1 g/dL; hematocrit, 30.5%; MCV, 90 μm3; platelet count, 89,000/mm3; and WBC count, 31,300/ mm3. From the peripheral blood smear shown in the figure, which of the following is the most likely diagnosis? A Acute lymphoblastic leukemia B Chronic lymphocytic leukemia C Infectious mononucleosis D Iron deficiency anemia E Leukemoid reaction
B Chronic lymphocytic leukemia CLL
248
24 A 62-year-old man has experienced vague abdominal discomfort accompanied by bloating and diarrhea for the past 6 months. On physical examination, there is a midabdominal firm mass. The stool is positive for occult blood. An abdominal CT scan shows an 11 × 4 cm mass involving the wall of the distal ileum and adjacent mesentery. A laparotomy is performed, and the mass is removed. Microscopically, the mass is composed of sheets of large lymphoid cells with large nuclei, prominent nucleoli, and frequent mitotic figures. The neoplastic cells mark with CD19+ and CD20+ and have the BCL6 gene rearrangement. Which of the following prognostic features is most applicable to this case? A Aggressive, can be cured by chemotherapy B Aggressive, often spreads to liver, spleen, and marrow C Aggressive, often transforms to acute leukemia D Indolent, can be cured by chemotherapy E Indolent, often undergoes spontaneous remission F Indolent, survival of 7 to 9 years without treatment
A Aggressive, can be cured by chemotherapy Diffuse large cell lymphoma of B cells
249
22 A 12-year-old boy has had increasing abdominal distention and pain for the past 3 days. Physical examination of his abdomen shows lower abdominal tenderness with tympany and reduced bowel sounds. An abdominal CT scan shows a 7-cm mass involving the region of the ileocecal valve. Surgery is performed and the resected mass microscopically shows sheets of intermediate-sized lymphoid cells, with nuclei having coarse chromatin, several nucleoli, and many mitotic figures. A bone marrow biopsy sample is negative for this cell population. Cytogenetic analysis of the cells from the mass shows a t(8;14) karyotype. Flow cytometric analysis reveals 40% of the cells are in S phase. The tumor shrinks dramatically after a course of chemotherapy. Which of the following is the most likely diagnosis? A Acute lymphoblastic leukemia/lymphoma B Burkitt lymphoma C Diffuse large B-cell lymphoma D Follicular lymphoma E Plasmacytoma
B Burkitt lymphoma Burkitt lymphoma Sporadic
250
25 A 9-year-old boy living in Uganda has had increasing pain and swelling on the right side of his face over the past 8 months. On physical examination, there is a large, nontender mass involving the mandible, which deforms the right side of his face. There is no lymphadenopathy or splenomegaly, and he is afebrile. A biopsy of the mass is performed and microscopic examination shows intermediate-sized lymphocytes with a high mitotic rate. A chromosome analysis shows a 46,XY,t(8;14) karyotype in these cells. The hemoglobin concentration is 13.2 g/dL, platelet count is 272,000/mm3, and WBC count is 5820/mm3. Infection with which of the following viruses is most likely to be causally related to the development of these findings? A Cytomegalovirus B Epstein-Barr virus C Hepatitis B virus D HIV E Human papillomavirus F Respiratory syncytial virus
B Epstein-Barr virus Endemic african variety of burkitt lymphoma
251
23 A 55-year-old man felt a lump near his shoulder 1 week ago. On physical examination, there is an enlarged, nontender, supraclavicular lymph node and enlargement of the Waldeyer ring of oropharyngeal lymphoid tissue. There is no hepatosplenomegaly. CBC is normal except for findings of mild anemia. A lymph node biopsy specimen shows replacement by a monomorphous population of lymphoid cells that are twice the size of normal lymphocytes, with enlarged nuclei and prominent nucleoli. Immunohistochemical staining and flow cytometry of the node indicates that most lymphoid cells are CD19+, CD10+, CD3−, CD15−, and terminal deoxynucleotidyl transferase negative (TdT−). A BCL6 gene mutation is present. Which of the following is the most likely diagnosis? A Acute lymphoblastic lymphoma B Chronic lymphadenitis C Diffuse large B-cell lymphoma D Hodgkin lymphoma E Small lymphocytic lymphoma
C Diffuse large B-cell lymphoma Diffuse B cell lymphoma
252
30 A 62-year-old man has had fever and a 4-kg weight loss over the past 6 months. On physical examination, his temperature is 38.6° C. He has generalized nontender lymphadenopathy, and the spleen tip is palpable. Laboratory studies show hemoglobin, 10.1 g/dL; hematocrit, 30.3%; platelet count, 140,000/mm3; and WBC count, 24,500/mm3 with 10% segmented neutrophils, 1% bands, 86% lymphocytes, and 3% monocytes. A cervical lymph node biopsy specimen microscopically shows a nodular pattern of small lymphoid cells. A bone marrow biopsy specimen shows infiltrates of similar small cells having surface immunoglobulins that are CD5+, but CD10−. Cytogenetic analysis indicates t(11;14) in these cells. What is the most likely diagnosis? A Acute lymphoblastic lymphoma B Burkitt lymphoma C Follicular lymphoma D Mantle cell lymphoma E Small lymphocytic lymphoma
D Mantle cell lymphoma Mantle cell lymphoma
253
42 A 33-year-old man has experienced multiple nosebleeds along with bleeding gums for the past month. On examination, his temperature is 37.3° C. He has multiple cutaneous ecchymoses. Laboratory studies show hemoglobin, 8.5 g/dL; hematocrit, 25.7%; platelet count, 13,000/mm3; and WBC count, 52,100/mm3 with 5% segmented neutrophils, 5% bands, 2% myelocytes, 83% blasts, 3% lymphocytes, and 2% monocytes. Examination of his peripheral blood smear shows the blasts have delicate nuclear chromatin along with fine cytoplasmic azurophilic granules. These blasts are CD33+. Which of the following morphologic findings is most likely to be present on his peripheral blood smear? A Auer rods B Döhle bodies C Hairy projections D Heinz bodies E Sickle cells F Toxic granulations
A Auer rods AML
254
43 A 38-year-old man experiences sudden onset of a severe headache. Physical examination shows no localizing neurologic signs and no organomegaly. A stool sample is positive for occult blood. Areas of purpura appear on the skin of his extremities. Laboratory studies show hemoglobin of 9.6 g/dL, hematocrit of 28.9%, platelet count of 16,400/mm3, and WBC count of 75,000/mm3. The peripheral blood smear has the appearance shown in the figure; schistocytes also are seen. The plasma D-dimer level (fibrin degradation products), prothrombin time, and partial thromboplastin time all are elevated. Cytogenetic analysis of cells from a bone marrow biopsy specimen is most likely to yield what karyotypic abnormality? A t(8;14) B t(8;21) C t(9;22) D t(14;18) E t(15;17)
E t(15;17) Acute promyelocytic leukemia (M3)
255
47 In an experiment, cell samples are collected from the bone marrow aspirates of patients who were diagnosed with lymphoproliferative disorders. Cytogenetic analyses are performed on these cells, and a subset of the cases is found to have the BCR-ABL fusion gene from the reciprocal translocation t(9;22)(q34;11). The presence of this gene results in increased tyrosine kinase activity. Patients with which of the following conditions are most likely to have this gene? A Acute promyelocytic leukemia B Chronic myelogenous leukemia C Follicular lymphoma D Hodgkin lymphoma, lymphocyte depletion type E Multiple myeloma
B Chronic myelogenous leukemia CML
256
49 A 60-year-old woman has had headaches and dizziness for the past 5 weeks. She has been taking omeprazole for ulcers. On physical examination, she is afebrile and normotensive, and her face has a plethoric to cyanotic appearance. There is mild splenomegaly, but no other abnormal findings. Laboratory studies show hemoglobin, 21.7 g/dL; hematocrit, 65%; platelet count, 400,000/mm3; and WBC count, 30,000/mm3 with 85% polymorphonuclear leukocytes, 10% lymphocytes, and 5% monocytes. The peripheral blood smear shows abnormally large platelets and nucleated RBCs. Which of the following is most characteristic of the natural history of this patient’s disease? A Development of a gastric non-Hodgkin lymphoma B Increase in monoclonal serum immunoglobulin C Marrow fibrosis with extramedullary hematopoiesis D Spontaneous remissions and relapses without treatment E Transformation into acute B-lymphoblastic leukemia
C Marrow fibrosis with extramedullary hematopoiesis Polycythemia vera (PCV)
257
50 A 45-year-old man has experienced a gradual weight loss and weakness, anorexia, and easy fatigability for 9 months. Physical examination shows marked splenomegaly. CBC shows hemoglobin, 12.9 g/dL; hematocrit, 38.1%; MCV, 92 μm3; platelet count, 410,000/mm3; and WBC count, 168,000/mm3. The peripheral blood smear is depicted in the figure. Karyotypic analysis shows the Ph1 chromosome. The patient undergoes chemotherapy with imatinib mesylate (tyrosine kinase inhibitor). He remains in remission for 3 years and then begins to experience fatigue and an 8-kg weight loss. CBC shows hemoglobin, 10.5 g/dL; hematocrit, 30%; platelet count, 60,000/μL; and WBC count, 40,000/μL. Karyotypic analysis shows two Ph1 chromosomes and aneuploidy. Flow cytometric analysis of the peripheral blood shows CD19+, CD10+, sIg−, and CD3− cells. Which of the following complications of the initial disease did this patient develop after therapy? A Acute myeloblastic leukemia B B-cell lymphoblastic leukemia C Hairy cell leukemia D Myelodysplastic syndrome E Sézary syndrome
B B-cell lymphoblastic leukemia CML
258
51 A 50-year-old man has had headache, dizziness, and fatigue for the past 3 months. His friends have been commenting about his increasingly ruddy complexion. He also has experienced generalized and severe pruritus, particularly when showering. He notes that his stools are dark. On physical examination, he is afebrile, and his blood pressure is 165/95 mm Hg. There is no hepatosplenomegaly or lymphadenopathy. A stool sample is positive for occult blood. CBC shows hemoglobin, 22.3 g/dL; hematocrit, 67.1%; MCV, 94 μm3; platelet count, 453,000/mm3; and WBC count, 7800/mm3. What is the most likely diagnosis? A Chronic myelogenous leukemia B Erythroleukemia C Essential thrombocytosis D Myelodysplastic syndrome E Polycythemia vera
E Polycythemia vera (PCV)
259
27 A 67-year-old man has had increasing weakness, fatigue, and weight loss over the past 5 months. He now has decreasing vision in both eyes and has headaches and dizziness. His hands are sensitive to cold. On physical examination, he has generalized lymphadenopathy and hepatosplenomegaly. Laboratory studies indicate a serum protein level of 15.5 g/dL and albumin concentration of 3.2 g/dL. A bone marrow biopsy is performed, and microscopic examination of the specimen shows infiltration by numerous small plasmacytoid lymphoid cells with Russell bodies in the cytoplasm. Which of the following additional laboratory findings is most likely to be reported for this patient? A Bence Jones proteinuria B Hypercalcemia C Karyotype with t(14;18) translocation D Monoclonal IgM spike in serum E WBC count of 255,000/mm3
D Monoclonal IgM spike in serum Lymphoplamacytic lymphoma (waldenstrom macroglobulinema) ``` Hyperviscosity Visual distrubances Dizziness Headache Raynaud ```
260
48 A 63-year-old woman experiences a burning sensation in her hands and feet. Two months ago, she had an episode of swelling with tenderness in the right leg, followed by dyspnea and right-sided chest pain. On physical examination, the spleen and liver now appear to be enlarged. CBC shows hemoglobin, 13.3 g/dL; hematocrit, 40.1%; MCV, 91 μm3; platelet count, 657,000/mm3; and WBC count, 17,400/mm3. The peripheral blood smear shows abnormally large platelets. Which of the following is the most likely diagnosis? A Acute myelogenous leukemia B Chronic myelogenous leukemia C Essential thrombocytosis D Myelofibrosis with myeloid metaplasia E Polycythemia vera
C Essential thrombocytosis
261
35 An 83-year-old man complains of worsening malaise and fatigue over the past 5 months. On physical examination, he is afebrile and normotensive. The spleen tip is palpable. A CBC shows hemoglobin, 10.6 g/dL; hematocrit, 29.8%; MCV, 92 μm3; platelet count, 95,000/mm3; and WBC count, 4900/mm3 with 63% segmented neutrophils, 7% bands, 2% metamyelocytes, 1% myelocytes, 22% lymphocytes, 5% monocytes, and 3 nucleated RBCs per 100 WBCs. The peripheral blood smear shows occasional teardrop cells. An examination of the bone marrow biopsy specimen and smear is most likely to show which of the following findings? A Erythroid hyperplasia B Extensive fibrosis C Fatty replacement D Many megaloblasts E Numerous myeloblasts
B Myelofibrosis Tear drop cells
262
52
A Myeofibrosis
263
Waldenstrom Macroglobulinema - proliferation - associated with - dont have
Monoclonal proliferation of IgM Assoc w/ - Amyloidosis - Hyperviscosity No lytic bone lesions
264
Myeloproliferative neoplasma 3 types mutation
Monclonal proliferation of mature myeloid cells Types - polycythemia vera - essential thrombocytosis - myelofibrosis Mutation - JAK2
265
JAK2 mutation in red cell precursor Increased red cell mass Decreased erythropoietin
Polycythemia vera
266
``` Red face Pruritis after hot shower Splenomegaly hyperviscosity of blood HA Red hands and feet ```
Polycythemia vera
267
Increased platelets | Low thrombopoietin
Essential thrombocytosis
268
Fibrosis and olbiteration of marrow space tear drop
Myelofibrosis
269
Monoclonal Ab spike
MM MGUS Waldenstrom macroglobulinemia
270
MM urine analysis
No protein in urine Monoclonal Ab spike on UPEP
271
A patient presents with hypertension, hypokalemia, metabolic alkalosis and low plasma renin. Diagnosis? Treatment
Primary hyperaldosteronism (Conn syndrome) Spironolactone
272
Stain for carcinoma
Cytokeratin stain
273
Epithelial vs mesenchymal tumors
Epithelial - adenoma - papilloma - carcinoma Mesenchymal - sarcoma - oma
274
Liver metastasis
"Cancer Sometime Penetrates Benign Liver" ``` Colon Stomach Pancreas Breast Lung ``` Colon >> stomach > pancreas ``` Elevated LFTs Abdominal pain Hepatomegaly Ascites Jaundice ```
275
Brain Metastasis
"Lots of Bad Stuff Kills Glia" ``` Lung Breast Skin (melanoma) Kidney GI tract ``` Lung > breast > melanoma, colon, kidney HA Neurological deficits Cognitive function Seizures Well circumscribed tumors at grey/white matter junctions
276
Bone Metastasis
"Permanently Relocated Tumors That Like Bone" ``` Prostate Renal cell cancer Testes Thyroid Lung Breast ``` Prostate, breast > lung, thyroid, kidney
277
Hypercalcemia of malignancy
Squamous cell lung cancer Secretes PTHrP Head and neck squamous cancers Breast cancer Renal cell carcinoma
278
Paraneoplastic of Small cell lung cancer
ACTH --> cushing syndrome ADH --> SIADH - hyponatremia Lambert eaton syn - antibodies against presynaptic calcium channel at NMJ - weakness improve with use Cerebellar degeneration
279
Psammoma bodies
Lamellated concentric calcific spheruloes Rings in a tree PSaMMoMa bodies - Papillary thyroid cancer - Serous papillary cystadenocarcinoma of the ovary - Malignant mesothelioma - Meningioma
280
What cancer is associated with Hashimoto thyroiditis
Marginal cell lymphoma
281
To which main two molecules is iron bound in human cells? To which two main molecules is iron bound in blood?
Human cells - Myoglobin - Ferritin Blood - Hemoglobin - Transferrin
282
Rb mutation - type of mutation - see in - sporadic vs inherited - Rb normal binds to
Tumor suppressor gene mutation TWO mutations Retinoblastoma Osteosarcoma White reflex in eye 60% sporadic (unilateral) 40% inherited (unilateral or bilateral) E2F
283
Mutation seen in more than 50% of human cancers
p53 causes cell cycle arrest normally
284
Mutation in APC - type mutation - seen in
Tumor suppressor genes Negative regulator of beta-catenin/WNT pathway Familial adenomatous polyposis Colon cancer
285
BRCA mutation | - type of mutation
Tumor suppressor genes - DNA repair protein BReast CAncer (only 3% have this mutation) Ovarian cancer
286
DPC mutation
Tumor suppressor protein Deleted in pancreatic cancer
287
DCC mutation
Tumor suppressor protein Deleted in colon cancer
288
NF1 mutation - type of mutation - chromosome - presentation
Tumor suppressor protein - Neurofibromin (Ras GTPase activating protein) NF type 1 Neurofibromas, cafe au lait spots Chr 17
289
NF2 mutation - type of mutation - chromosome - presentation
Tumor suppressor protein - Merlin (schwannomin) protein NF type 2 Bilateral acoustic schwannoma chr 22
290
WT1 WT2 mutation on what chromosome type of mutation
Tumor suppressor protein chr 11
291
Von Hippel Lindau mutation on what chromosome type of mutation
Tumor suppressor protein Chr 3
292
Most common mutations
p53 and RAS
293
RAS mutation
oncogene mutation 1 mutated allele
294
K-RAS mutation - type - seen in
oncogene mutation Colon Lung Pancreatic tumors
295
H-Ras mutation - type - seen in
oncogene mutation Bladder and kidney tumors
296
N-RAS mutation - type - seen in
oncogene mutation Melanomas Hematologic malignancies Follicular thyroid cancer
297
BCR-ABL - type - normal fxn - what occurs - seen in
oncogene mutation - tyrosine kinase ABL codes for tyrosine kinase ABL translocated from 9 to 22 Philadelphia chromosome CML
298
RET mutation - type mutation - associated with
oncogene mutation - receptor tyrosine kinase MEN 2A and 2B - Pheochromocytoma - Medullary thyroid cancer Papillary carcinoma of thyroid
299
HER2/neu mutation - type mutation - seen in
oncogene mutation - receptor tyrosine kinase ERBB2 Breast cancer Ovarian cancer Gastric cancer
300
c-myc - type mutation - seen in
oncogene mutation - transcription factor burkitt lymphoma
301
L-myc
Lung tumors
302
N-myc
Neuroblastoma (adrenal medulla)
303
What is the most common urea cycle disorder
Ornithien transcarbamylase deficiency
304
Aflatoxins
Toxins produced by aspergillus mold Causes mutation in p53
305
Nitrosamines - found in - assoc with
Found in smoked foods Assoc w/ - Gastric cancer - Squamous cell esophageal cancer
306
Arsenic causes
Lung cancer Squamous cell skin cancer angiosarcoma- Liver
307
Vinyl chloride causes
angiosarcoma- liver
308
Alkylating agents assoc with
Leukemia | Lymphoma
309
Benzene assoc with
Leukemia | Hodgkin lymphoma
310
Naphthalene causes
Transitional cell bladder cancer
311
HHV-8
Kaposi sarcoma
312
EBV associated iwth
Hodgkin lymphoma (Reed-sternberg cells) Burkitt lymphoma (starry sky, 8;14) Nasopharyngeal carcinoma
313
Streptococcus bovis assoc with
Colon cancer
314
Schistosoma haematobium assoc with
Squamous cell cancer of bladder
315
Clonorchis sinensis assoc with
Biliary cancer
316
Tuberous sclerosis associated with what neoplasms
Cardiac rhabdomyoma Renal angiomyolipoma Astrocytoma
317
Ionizing radiation associated with what neoplasms
AML CML Papillary thyroid cancer
318
What UV radiation most problematic
UVB
319
What is the RL enzyme in purine synthesis? RL enzyme in pyrimidine synthesis?
Purine: Glutamine PRPP amidotransferase Pyrimidine: Carbamoyl phosphate synthase 2
320
PSA tumor marker
Prostate cancer
321
CA-125
Ovarian cancer
322
Alkaline phosphatase
bone metastasis Biliary disease paget disease of bone
323
alpha-fetoprotein
hepatocellular carcinoma | Testicular tumors
324
CA 19-9
pancreatic cancer
325
CEA Calcitonin Chromogranin
Colon Pancreatic cancer Medullary thyroid cancer (alone and with MEN2A, MEN2B) Neuroendocrine tumors
326
S-100
Melanoma | Schwannoma
327
Tartrate-resistant acid phosphatase (TRAP)
Hairy cell leukemia
328
Which tumor marker would you use to monitor each? 1. Hepatocellular carcinoma 2. Ovarian cancer 3. Pancreatic cancer 4. Melanoma 5. Colon cancer
1. AFP 2. CA-125 3. CA 19-9, CEA 4. S-100 5. CEA
329
RL enzymes for each? 1. Urea cycle 2. Hexose monophosphate pathway 3. Fatty acid synthesis 4. Beta-oxidation of fatty acids 5. Ketone body synthesis 6. Cholesterol synthesis 7. Bile acid synthesis 8. Heme synthesis
1. Carbamoyl phosphate synthetase I 2. Glucose 6 phosphate dehydrogenase 3. Acetyl CoA carboxylase 4. Carnitine acyltransferase 5. HMG CoA synthase 6. HMG CoA reductase 7. 7-alpha hydroxylase 8. Aminolevulinate synthase
330
What are the irreversible enzymes involved in gluconeogenesis?
Pyruvate carboxylase (pyruvate --> oxaloacetate) PEP carboxykinase (oxaloacetate --> phosphoenolpyruvate) Fructose 1,6 bisphosphatase (Fructose 1,6- bisphosphate --> Fructose 6 phosphate) Glucose 6 phosphate (Glucose 6 phosphate --> glucose)
331
Enveloped viruses spread
Droplets perientrally or sexually
332
Non-enveloped viruses spread
Fecal oral spread
333
Non-enveloped RNA viruses
"my Real Heavy Picture of California was Ruined without an Envelope" Reovirus Hepevirus Picornavirus Calicivirus
334
Non-enveloped DNA viruses
"PaPa and Aunt Poly didnt have an Envelop" Parvovirus Papillomavirus Adenovirus Polyomavirus
335
DNA viruses typically replicate
In the nucleus
336
RNA viruses typically replicate
in the cytoplasm
337
DNA viruses that replicate in the cytoplasm
Smallpox virus | Molluscum contagiosum
338
RNA viruses that replicate in the nucleus
Influenza virus | Retroviruses
339
Viral recombination where two viruses exchange entire segment - example - only what
Viral reassortment RNA viruses only Influenza virus
340
When two different viruses infect same host a genes are exchanged from two different chromosomes
Viral recombination
341
When two viruses infect a cell and one is non-functional so other virus makes a functional protein that serves both viruses
Viral Complementation
342
When two viruses infect the same host and the progeny receive surface proteins from either parent
Phenotypic mixing
343
Ploidy of viruses
All RNA and DNA viruses are haploid Except: retroviruses (2) of ssRNA
344
DNA viruses - examples - typical features - exceptions
HAPPy HiPPo Dung ``` Herpesvirus Adenovirus Parvovirus Papillomavirus Hepadnavirus Polyomavirus Poxvirus DNA ``` Linear dsDNA Icosahedral shape Replicate in nucleus Only parvoviridae (ssDNA) Papillomarvirus (circular) Polyomavirus (circular) Hepadnavirus (circular)
345
RNA viral genomes
(+) stranded RNA viruses I went to a RETRO TOGA party, where I drank FLAvored CORONA and ate HIPPIE CALIfornia PICkles ``` Retrovirus Togavirus Flavivirus Coronavirus Hepevirus Calicivirus Picornavirus ``` All ssRNA except reoviridae (dsRNA)
346
Negative-sense ssRNA
Not immediately translated Can only be transcribed and not directly translated Bring along viral transcriptase Always Bring Polymerase Or Fail Replication ``` Arenaviruses Bunyaviruses Paramyxoviruses Orthomyxoviruses Filoviruses Rhabdoviruses ```
347
dsRNA brings along what
Polymerase
348
Live attenuated vaccine
"Attention Teachers" Please Vaccinate Small Beautiful Young Infants with MMR Regularly ``` Adenovirus Typhoid Polio (Oral) Varicella Small pox BCG Yellow fever Influena (intranasal) MMR Rotavirus ```
349
Killed vaccine
RIP Always Rabies Influenza (injection) Polio (injectable) Hepatitis A
350
Subunit vaccines
Use specific viral antigens to stimulate immune response hepatitis B HPV
351
Egg allergy
Yellow fever Influenza (MMR)
352
When should live virus vaccines be considered for HIV positive patients
All infants with HIV - Rotavirus vaccine CD4 count > 200 - MMR - Varicella vaccine Not recommended - Live virus influenza vaccine - Herpes zoster vaccine
353
What condition is suggested by each of the following 1. Signet ring cells 2. Nutmeg liver 3. RBC casts in urine
1. Gastric adenocarcinoma, lobular carcinoma in situ of breast 2. Right sided heart failure and budd-chiari 3. Acute glomerulonephritis
354
Infects spindle cells
HHV-8 Kaposi sarcoma associated herpesvirus (KSHV)
355
Temporal lobe encephalitis
Herpes simplex virus 1 (HSV-1)
356
Tzanck smear
Multinucleated giant cells positive HSV-1, HSV-2, VZV Low specificity Low sensitivity
357
Intranuclear eosinophilic inclusions
Type A Cowdry bodies | HSV, VZV, CMV
358
EBV infects what cells causes what cell type seen
Infects B cells Causes T cell response Downey cells - Foamy appearing basophilic cytoplasm
359
Foamy appearing cells with basophilic cytoplasm
Downey cells T cells EBV
360
Fever Sorethroat LAD of posterior cervical nodes Splenomegaly (+) monospot test
Infectious mononucleosis EBV
361
Ab against antigens found on horse, sheep and beef RBCs
Monospot test Detects heterophiles EBV
362
Heterophile (-) mononucleosis
CMV infection | Acute HIV
363
Owl eye nuclear inclusions
CMV
364
Retinitis blind within days
CMV w/ AIDS
365
HIgh fever (102) 3-5 days Fever breaks Maculopapular rash all over body Seizures
Roseola Sixth disease Human Herpes virus -6
366
What group of patients is prone to esopahgitis caused by CMV
HIV patients (CD4 < 50)
367
Hypoglycmeia Jaundice Cirrhosis
Fructose intolerance
368
Child with bright red rash on cheeks
``` Slap cheek rash Erythema infectiosum (fifth disease) ``` Parvovirus B19
369
Upper respiratory infection Then conjunctivitis Diarrhea
Adenovirus | dsDNA virus
370
Variola poxvirus vs vaccinia pox virus
Variola poxvirus - smallpox Vaccinia poxvirus - cowpox
371
Flesh colored dome shaped lesions with central dimple (umbilication)
Molluscum contagiosum In immunocompetent will resolve in less than a year
372
Demyelination of CNA due to destruction of oligodendrocytes AIDS Progressive Visial symptoms
Progressive Multifocal leukoencephalopathy JC virus POlyomavirus
373
Only Hepatitis virus dsDNA
Hepatitis B virus
374
Hepatitis B virus is what type of virus
Hepadnavirus
375
Hepatitis B virus replication
dsDNA virus Partially double-stranded circular DNA enter nucleus Host polymerase makes RNA intermediate from dNA Viral reverse transcriptase makes DNA from RNA intermediate
376
Hides in sensory ganglia of S2 S3 Hides in trigeminal ganglia Hides in dorsal root ganglia
HSV-2 HSV-1 VZV
377
Opacities seen on X ray on both sides of carina
Bilateral hilar lymphadenopathy Sarcoidosis
378
Common cold
Rhinovirus | Coronavirus
379
``` Severe diarrhea and vomiting Winter months Daycare center Non-enveloped dsRNA virus ```
Rotavirus | type of reovirus
380
Western US/ canada Hiker, Fisherman, camping Flu- like
Coltivirus (type of reovirus) Self limiting
381
Types of picornaviruses
Enterovirus - Poliovirus - Coxsackievirus - Echovirus - Hepatitis A virus Rhinovirus
382
Types of enteroviruses
Enterovirus falls under Picornaviruses Spread through enteric system 4 types - Poliovirus - Coxsackievirus - Echovirus - Hepatitis A virus
383
Poliovirus
Infects grey matter of the anterior horn of the spinal cord and motor neurons of the pons of the medulla Causes paralysis
384
Aseptic meningitis Myocarditis URIs Summer -Diagnosis?
Echovirus PCR of CSF
385
Hand foot and mouth disease
Coxsackievirus
386
Most common cause of viral myocarditis in US
Coxsackievirus ``` Dilation of all chambers Dyspnea Chest pain Fever Arrhythmias ```
387
Mouth blisters, fever
herpangina Coxsackievirus
388
Coxsackievirus can cause
``` Myocarditis Aseptic meningitis Pericarditis herpangina (blisters mouth and fever) Hand, foot and mouth disease Febrile pharngitis ```
389
Picornavirus that is not an enterovirus
Rhinovirus Common cold
390
Vomiting and diarrhea | Cruise ship
Norovirus (caliciviruses) Fecal oral route
391
Types of flaviviruses
Yellow fever Dengue fever West nile virus St. Louis Encephalitis virus Most are arboviruses (spread by arthropod)
392
``` High fever, HA Epistaxis Gum bleeding petechial and purpura Black vomit Jaundice Africa ``` Spread by?
Yellow Fever Aedes mosquitoes Liver damage
393
Fever Severe musculoskeletal pain Retro-orbital headache Spread by? Can get? Test?
Dengue fever Mosquito Hemorrhagic fever Tourniquet test - field test for hemorrhagic fever - BP inflated leave 5 min - Excess petechiae
394
``` Headache Malaise Backpain Myalgia Anorexia Alteration in consciousness Neck stiffness ```
West Nile virus (WNV) Mosquito Serology for IgM anti-WNV antibodies Tx supportive
395
A 26-year-old woman has headaches for 4 weeks along with increasing malaise. Physical examination yields no remarkable findings. CT scan of the head shows no abnormalities. A lumbar puncture yields clear, colorless CSF with a normal opening pressure. Laboratory analysis of the CSF shows a normal glucose concentration and a minimally increased protein level. A few lymphocytes are present, but there are no neutrophils. A Gram stain and India ink preparation of the CSF are negative. Her condition gradually improves over the next 6 months. Serum serologic tests are most likely to show an elevated titer of antibodies to which of the following infectious agents? A Cryptococcus neoformans B Echovirus C Listeria monocytogenes D Neisseria meningitidis E Toxoplasma gondii
B. Echovirus Acute lymphocytic meningitis West nile Echovirus
396
Togaviruses
Rubella virus
397
Fever Lymphadenopathy Arthralgia Macuopapular rash
Rubella or german measles
398
Congenital rubella
Cardiac defects - PDA - Pulmonic stenosis Cataracts Deafness
399
11 A 6-year-old girl has a blotchy, reddish-brown rash on her face, trunk, and proximal extremities that developed over the course of 3 days. On physical examination, she has 0.2-cm to 0.5-cm ulcerated lesions on the oral cavity mucosa and generalized tender lymphadenopathy. A cough with minimal sputum production becomes progressively worse over the next 3 days. Which of the following viruses is most likely to produce these findings? A Epstein-Barr B Mumps C Rubella D Rubeola E Varicella zoster
D Rubeola Measles (rubeola) Rash Koplik spots Rubella: german measles, is a much milder infection than rubeola
400
7 term infant has initial Apgar scores of 8 and 10 at 1 and 5 minutes. On auscultation of the chest, a heart murmur is audible. There is hepatosplenomegaly. Cataracts of the crystalline lens are noted. The infant is at the 30th percentile for height and weight. Echocardiography shows a patent ductus arteriosus. Which of the following events is the most likely risk factor for the findings in this infant? A Congenital rubella infection B Dietary folate deficiency C Dispermy at conception D Erythroblastosis fetalis E Maternal thalidomide use F Paternal meiotic nondisjunction
A Rubella in first trimester
401
38 A family who recently emigrated from Romania brings their 7-year-old child to the pediatrician with complaints of conjunctivitis and periorbital swelling. The child has had coughing with a runny nose and high fever for 3 days. Small lesions with blue-white centers are seen in his oral cavity. Which of the following is the most likely cause of this child’s symptoms? (A) Diphtheria (B) Pertussis (C) Roseola (D) Rubella (E) Rubeola
E Rubeola
402
Retroviruses
Use reverse transcriptase HIV Human T cell Luekemia virus (HTLV)
403
What is the number one cause of fatal infantile gastroenteritis
Rotavirus
404
Posterior cervical adenopathy
EBV mononucleosis Cat scratch disease (bartonella) Acute otitis media
405
Avian influenza
H5N1 Spread only from bird to human 60% mortality Severe rapidly progressive couse Fever cough myalgias diarrhea, pancytopenia Tx: Oseltamivir
406
Swine flu
H1N1 Two swine strain One human strain One avian strain Flu and GI symptoms Tx: Oseltamivir or zanamivir
407
Influenza virus - type of virus - description of virus - two key features
Orthomyxoviruses Enveloped ssRNA Hemagglutinin - viral attachment to host cell Neuraminidase - release progeny virions
408
Barking seal cough child
Croup Parainfluenza virus - enveloped
409
Steeple sign
Croup Parainfluenza virus - enveloped
410
Winter months viruses
Influenza Rotavirus RSV
411
Summer months enterovirus
Poliovirus Echovirus Coxsackievirus
412
Summer months arbovirus
West Nile virus
413
Bronchiolitis Child Pneumonia in child
Infection of bronchioles RSV
414
Primary cause of penumonia in children
RSV
415
RSV virus has what
Transmembrane protein called Fusion protein (F protein) This allows infected cells to bond to near by uninfected cells Multinucleated giant cells
416
55 A 3-year-old boy has had a cough, headache, and slight fever for 5 days. He is now having increasing respiratory difficulty. On physical examination, his temperature is 37.8° C, pulse is 81/min, respirations are 25/min, and blood pressure is 90/55 mm Hg. On auscultation, there are inspiratory crackles, but no dullness to percussion or tympany. Respiratory syncytial virus is isolated from a sputum sample. Which of the following chest radiographic patterns is most likely to be present? A Hilar lymphadenopathy B Hyperinflation C Interstitial infiltrates D Lobar consolidation E Pleural effusions F Upper lobe cavitation
C Interstitial infiltrates RSV
417
Paramyxoviruses
RSV Parainfluenza virus Rubeola virus (measles) Mumps
418
Runny nose cough Conjunctivitis Blue grey specks on buccal mucosa surround by base of red Wide spread maculopapular rash that started on head and spread
Measles Enveloped Koplik spots
419
Parotits Orchitis Meningitis
Mumps enveloped Inflammation of parotid gland
420
Viral meningitis
Coxsackievirus Echovirus Enterovirus Mumps virus
421
``` Negri bodies Fever Nausea vomiting Strange behavior Hallucinations Hydrophobia Coma Death ```
Rabies virus (Rhabdovirus) Enveloped RNA
422
Eosinophilic cytoplasmic inclusions that contain viral nucleocapsids
Negri bodies | Rabies
423
Bullet shaped capsid
Rabies
424
Fever Progressive pulmonary edema Respiratory failure Exposure to mouse urine
Hantavirus
425
A 27-year-old man dies of acute respiratory distress syndrome 1 day after presenting to the hospital with shortness of breath and a fever of 38° C (100.4° F). On the second hospital day, he developed extreme pulmonary edema and hypotension before he died. His family says that he had recently gone hiking and caving in an area known to be heavily populated with rodents. Which of the following is the most likely cause of death? (A) Dengue virus (B) Ebola virus (C) Hantavirus (D) Marburg virus (E) Rhabdovirus
C. Hantavirus
426
Illnesses spread through animal urine
Hantavirus Lassa virus LCM virus Leptospirosis
427
``` Fever HA myalgias Followed by vomiting and diarrhea Multiorgan failure Shock Death ```
Ebola virus
428
A patient present with fever, vomiting and diarrhea. She was in Sierra Leone two weeks visiting family. What viral infection? Type of isolation
Ebola Contact and droplet precautions
429
Viral proteins of HIV
Gp120 | Gp41
430
Gp120
Grabs onto CD4 surface receptor on T cells and macrophages Once connected, changes shape and allows it to bind to coreceptor (CCR5 or CXCR4)
431
Tropsim
Which coreceptor HIV uses to enter cell only CXCR4: X4 viruses Only CCR5: R5 viruses Both : dual tropism
432
GP41
Fusion and entry into host cells
433
Three main structural genes in HIV genoma
Gag: encodes p24 capsid protein Env: gp120 and gp41 Pol: codes for reverse transcriptase
434
Diagnosis of HIV check
P24 antigen adn HIV antibodies Viral load (PCR test)
435
AIDS diagnosis
CD4 < 200 < 14% lymphocytes are Cd4 AIDs defining condition - Pneumocystis jirovecii pneumonia
436
Ring enhancing lesions
HIV associated Primary CNS lymphoma (solitary lesion) Toxoplasmosis (multiple lesions)
437
HIV < 200 opportunistic infections
Pneumocystis jirovecii pneumonia
438
HIV < 100 opportunistic infections
Histoplasmosis capsulatum Candida albicans
439
HIV < 50 opportunistic infections
Mycobacterium tuberculosis Mycobacterium avium complex CMV retinitis
440
Hepatosplenomegaly Fever Cough CD4 < 150
Histoplasmosis capsulatum
441
Chronic watery diarrhea HIV patient diagnosis
Cryptosporidium spp. (c.parvum) Oocytes on modified acid-fast stain of stool turns bright pink or red
442
HIV patient covered in what look likes cherry angiomas
Bacillary angiomatosis | Bartonella henselae
443
What four molds are considered dermatophytes (fungal species that invade superficial skin)
Trichophyton Microsporum Epidermophyton Malassezia furfur
444
Diaper rash | Satellite lesions
Candida albicans Shiny skin or eroded
445
Blood culture | Germ tubes have small projections on side of cell
Candida albicans
446
Narrow based budding yeast
Cryptococcal neoformans
447
India ink | Clear circle
Cryptococcal neoformatns
448
Soap bubble lesion on imaging
Cryptococcal meningoencephalitis
449
Silver stain Ground glass appearance of interstital infiltrates
Pneumocystis jirovecii (PCP)
450
Narrow septate hyphae that branch at acute angles (45 degrees)
Aspergillus fumigatus
451
Broad irregularly shaped, nonseptate hyphae branching at right angles (90 degrees)
Mucor and Rhizopus
452
``` Facial pain HA Large necrotic lesion, black escar Blindness CN deficits ```
Rhinocerebral mucomycosis
453
Most common type of dermatophytes with animal reservoir
Microsporium (dog or cat)
454
Hypopigmented or hyperpigmented lesion on body Scaling plaque Yeast
Tinea versicolor Malassezia furfur
455
KOH prep spaghetti and meatball appearance
Tinea versicolor | Malassezia furfur
456
What is associated iwth each clue 1. Cat scratch 2. Cat bite 3. Cat feces 4. Puppy feces 5. Animal urine
1. Bartonella henselae 2. Pasteurella multocida 3. Toxoplasma 4. Yersinia enterocolitica, campylobacter jejuni 5. Leptospira, hantavirus
457
Leptin on hypothalamus
Inhibits lateral area and stimulates ventromedial area of hypothalamus = satiety
458
Organisms associated with birds
``` Histoplasma capsulatum Cryptococcus neoformans Chlamydophilia psittaci H5N1 influenza West Nile virus ```
459
Mississippi ohio river valley Bird and bat droppings Spelunkers - Imaging - what happens to it
Histoplasma capsulatum Enters through lung Engulfed by macrophages Hyphae and spores on imaging Macrophage filled w/ 100 spores
460
Mississippi and ohio river basins Thick refractile wall around yeast broad based budding
Blastomyces dermatitidis Granulomatous nodules
461
Southwestern U.S Mexico Pneumonia Spherule -filled with endospores
Coccidioides immitis
462
A 46-year-old man from northern Mexico has had fever, nonproductive cough, and weight loss for 2 months. On examination his temperature is 37.5 ° C. A chest radiograph shows a miliary pattern of small nodules in all lung fields. Bronchoalveolar lavage is performed and microscopic examination of the fluid shows organisms averaging 50 microns in diameter with thick walls and filled with endospores. Which of the following infections is he most likely to have? A Blastomycosis B Coccidioidomycosis C Histoplasmosis D Mycobacteriosis E Nocardiosis F Paracoccidioidomycosis
B. Coccidioidomycosis
463
Pneumonia Granulomatos changes in mucous membranes Captain wheel Latin america
Paracoccidioides brasiliensis Paracocci parasails with a captains wheel all the way to Latin america
464
Gardner Dimorphic fungus Pustule on puncture site Nodules follow draining lymphatics Tx
Sporothrix schenckii Tx: Itraconazole (first line) potassium iodine
465
Mold form contains barrel shaped arthroconidia
Coccidioides immitis
466
Associated iwth dust storms
Coccidioides immitis
467
Multiple budding of yeast form
Paracoccidioides brasillensis
468
Councilman bodies
Apoptotic liver cells Viral hepatits yellow fever
469
Acute fatty foul smelling diarrhea Abdominal bloating - Diagnosis Tx
Giardia lamblia Pear-shaped trophozoites or cysts in stool Metronidazole Tinidazole
470
Pear-shaped trophozoites
Giardia lamblia
471
Bloody diarrhea Flask shaped ulcers RUQ pain - type organism - transmittered - diagnosis - tx
Entamoeba histolytica Liver abscesses Fecal-oral route - eating food or water contaminated with feces that contain cysts Trophozoites or cysts in stool Treatment - trophozoites= metronidazole or tinidazole - cysts= iodoquinol or paromomycin
472
Foul smelling vaginal discharge Motile -transmission - wet mount - vaginal pH - tx -
Trichomonas vaginalis Sexual transmission Pear shaped appearance Increase vaginal pH Metronidazole
473
Ring enhancing lesions in brain
Toxoplasma gondii
474
Chorioretinitis Hydrocephalus Intracranial calcifications
Toxoplasma gondii
475
Toxoplasma gondii acquired how
Cat feces or litter | Eating cyst in infected meat
476
Rapidly fatal meningoencephaltiis Swimming
Naegleria fowleri Swimming in freshwater lakes Enters nose passes through cribriform plate
477
A 40-year-old man goes on a camping vacation with his family. One day after swimming in a freshwater lake near the camp site, he develops nausea and vomiting and starts to behave irrationally. His family takes him to the emergency department, where blood samples are taken and a spinal tap is performed. He is diagnosed with a rapidly progressing meningoencephalitis and dies shortly thereafter. Which of the following protozoa was most likely the cause of the man’s illness? (A) Cryptosporidium species (B) Entamoeba histolytica (C) Leishmania donovani (D) Naegleria fowleri (E) Plasmodium falciparum
(D) Naegleria fowleri
478
55 A 9-year-old child who is living in a mud hut in Paraguay has a sore persisting on her face for 4 days. Physical examination shows an indurated area of erythema and swelling just lateral to the left eye, accompanied by posterior cervical lymphadenopathy. She has unilateral painless edema of the palpebrae and periocular tissues. Two days later, she has malaise, fever, anorexia, and edema of the face and lower extremities. On physical examination 1 week later, there is hepatosplenomegaly and generalized lymphadenopathy. Which of the following pathologic findings is most likely to develop in this patient? A Cerebral abscesses B Chronic arthritis C Dilated cardiomyopathy D Meningitis E Mucocutaneous ulcers F Paranasal bony destruction
C Dilated cardiomyopathy Trypanosoma cruzi
479
Trypanosoma brucei spp
African sleeping sickness tsetse fly Fever Enlarged LN Sleepiness Encephaltiis Coma and death Blood smear: long whispy organisms in blood Early: Suramin CNS involved: Melarsoprol
480
Dilated cardiomyopathy Megesophagus Megacolon
Trypanosoma cruzi Chagas disease Reduviid bug - painless bit - Feces scrated into skin Benznidazole Nifurtimox
481
Spiking fevers Hepatosplenomegaly Pancytopenia - transmission - diagnosis - tx
Leishmania donovani Visceral leishmaniasis Sandfly Amastigotes inside macrophages Liposomal amphotericin B
482
Ulcerating papules that are slow to heal
Leishmania donovani - Cutaneous leishmaniasis Sandyfly Sodium stibogluconate
483
Cyclic fever Headache Anemia Splenomegaly
Malaria Plasmodium species Mosquito
484
Malaria fever due to
RBCs rupture and release merozoites
485
Malaria that fever spikes every 48 hrs Every 72 hrs
48 hr - P. ovale - P. vivax 72 hr - P. malariae
486
Malaria - subtype - tx - Diagnosis
Plasmodium species P. vivax and P. ovale - dormant infection in liver Tx primaquine ``` P. falciparum - most severe - no dormant form - sequestration of RBCs --> occlusion of small vessels Tx: Chloroquine ``` Blood smear - Merozoites - Trophozoite= diamond ring - Gametocytes: banana shaped
487
Two ring forms inside one erythrocyte
Trophozoite form Babesia Microti
488
Maltese cross
inside RBC merozoites Babesia Microti
489
Babesia Microti - symptoms - blood smear - transmission - tx
Fever Hemolytic anemia Two rings inside RBC Maltese cross Ixodes tick coinfection w/ lyme disease Tx Quinine + Clindamycin
490
Names of following stages of malaria life cycle 1. Looks like diamond ring 2. Ruptures the cell host 3. Replicating intracellularly 4. Form injected from the anopheles mosquito 5. banana shaped
1. Early trophozoite 2. Merozoite 3. Schizont 4. Sporozoite 5. Gametocyte
491
Ingest helminths
"EAT" Enterobius vermicularis Ascaris lumbricoides Trichinella spiralis
492
Penetrating helminths
"Sand" Stronglyoides Ancylostoma Necator D
493
Worm lays eggs at anus tx
Enterobius vermicularis Nematode (round worm) Benzimidazoles - Albendazole - mebendazole Pyrantel pamoate
494
Giant round worm Eosinophilic pneumonitis - diagnosis - tx
Ascaris lumbricoides ``` Ingest eggs Hatch in intestines Release larvae penetrate bowel --> lungs Migrate up trachea and swallowed Mature worms in intestine ``` Eggs released in stool Benzimidazoles - Albendazole - mebendazole Pyrantel pamoate
495
Myositis Fever Eosinophilia Periorbital edema - acquired - diagnosis - tx
Trichinella spiralis Undercooked meat GI --> muscles Muscle biopsy: cysts with larvae in muscle tissues Bendzimidazoles
496
A 28-year-old woman from rural Guyana with a history of rheumatoid arthritis develops painful swelling of her hands and feet. She is treated with corticosteroid therapy. A month later, she develops profuse, watery diarrhea along with fever and cough. On examination, she has a temperature of 37.3° C. Laboratory studies show WBC count, 12,900/ mm3; and the WBC differential count shows 57% segmented neutrophils, 5% bands, 16% lymphocytes, 8% monocytes, and 14% eosinophils. Microscopic examination of a stool specimen shows ova and small rhabditoid larvae. Similar larvae are present in a sputum specimen. Which of the following infectious diseases is most likely to produce these findings? A Cysticercosis B Onchocerciasis C Schistosomiasis D Strongyloidiasis E Trichinosis
D. Strongyloidiasis
497
Rhabditiform larvae in feces tx
Strongyloides Threadworm Ivermectin Albendazole
498
Wound on feet Anemia Abdominal discomfort tx
Ancylostoma Necator Hook worms Benzimidazoles - Albendazole - mebendazole Pyrantel pamoate
499
Taenia solium - transmission - symptoms - tx
Cestodes (tapoworms) Ingestion of alrvae in undercooked port - GI tract infection by adult worm Ingestion of eggs in contaminated food or water - brain filled w/ cysts Tx Albendazole Praziquentel Dexamethaone: keep CNS symptoms from getting worse
500
Cestodes
Tape worms Taenia solium Diphyllobothrium latum Echinococcus granulosus
501
Brain filled with cysts | Seizures
Taenia solium Ingestion of eggs in contaminated food
502
Giant limbs - type - transmitted - tx
Wuchereria bancrofti Helminth, neomatoid Round worm in GI Mosquitoes Migrate to lymphatics Elephantiasis of limbs Hydrocele of scrotum Tx Diethylcarbamazine
503
Raw fish Megaloblastic anemia -def tx
Diphyllobothrium latum B12 deficiency Praziquantel
504
Hydatid cyst lesion in liver RUQ pain Well circumscribed circular lesion - transmitted - can find where in body - symptom - tx
Echinococcus granulosus dog feces Form mature larval cysts in liver and lungs Anaphylaxis Surgical removal of cysts
505
A 23-year-old man presents to the physician with abdominal distention and tenderness with no vomiting or diarrhea. Physical examination shows hepatosplenomegaly. Bowel sounds are normal. On questioning, the patient says that he traveled to eastern South America 1 year ago. Several weeks after returning from his trip, he remembers having fever, diarrhea, weight loss, and “funny looking stools.” Ultrasonography shows ascites and hepatic periportal fi brosis. Which of the following is most likely responsible for this patient’s present symptoms? (A) Appendicitis (B) Bowel obstruction (C) Enterocolitis (D) Portal hypertension (E) Ruptured viscus
D. Portal hypertension Schistosoma mansoni
506
A 29-year-old man has had hematuria for the past month. On physical examination, he is afebrile. There is diffuse lower abdominal tenderness, but no palpable masses. An abdominal radiograph shows a small bladder outlined by a rim of calcification. Cystoscopy is performed, and the entire bladder mucosa is erythematous and granular. Biopsy samples are taken. Which of the following histologic findings is most likely to be seen in these samples? A Acid-fast bacilli of Mycobacterium avium complex B Eggs of Schistosoma haematobium C Larvae of Trichinella spiralis D Migrating Ascaris lumbricoides E Taenia solium cysts
B. Eggs of schistosoma haematobium
507
Deals with snails
Schistosoma
508
Portal HTN Splenomegaly Egyptian immigrant tx
Schistosoma - blood fluke Praziquantel
509
Chronic bronchitis Hemoptysis acquired -t x
Paragonimus westermani Adult fluke in lung Undercooked crab meat Praziquantel
510
Pigmented gallstones Cholangiocarcinoma - transmission - lives - Tx
Clonorchis sinensis - liver fluke undercooked fish Lives in biliary tract Praziquantel Albendazole
511
Swimmers itch
Schistosoma
512
Fluke associated iwth hematuria and bladder cancer
Schistosoma haematobium
513
Burns all over the body what will occur over the 7 day?
Increased metabolic rate Not - Increased extracellular volume - Not increase in serum cholesterol concentration - Not loss of B vitamins in skin
514
Testicular tumor with friend egg appearance
Seminoma Germ cell tumor
515
Umbilical cord compression of infant, monitoring shows a normal fetal heart rate of 150/ min with variable spontaneous decelerations to 110/min. Why the decrease in fetal heart rate
Increased fetal systemic vascular resistance Compressing vessel, decreases radius increase in resistance Cause reflex bradycardia
516
20 yr history of T1D uses insulin why hypoglycemia
Impaired release of glucagon
517
Subscapularis does what
internal rotation
518
Parathyroid effects on parts of kidney
Increase reabsorption of Ca in distal convoluted tubule Decreased reabsorption of phosphate in proximal convoluted tubule Increase 1,25 OH2 D3 production by stimulating kidney in 1alpha-hydroxylase in PCT
519
Transfusion reactions
Type II hypersensitivity
520
Bone conduction > Air conduction
Conductive hearing loss Otosclerossi
521
Pregnancy, pituitary enlargement
Lactotroph hyperplasia due to estrogen increase
522
Lupus - type of rxn - deficiency of
type II hypersensitivity C1q, C4, C2
523
Genetic component to hypertrophic obstructive cardiomyopathy
Mutations in sarcometric proteins - myosin binding protein C - beta-myosin heavy chains
524
Papilledema increased
HTN Increased angiotensin
525
Primary adrenal insufficiency labs
Hyponatremia Hyperkalemia Metabolic acidosis Hyperkalemia leads to K entering all cells (via H/K exchanger in exchange for H exiting cells)
526
Fixed split S2 | Systolic murmur at left sternal border
Atrial septal defect
527
Panic attack what will the labs show
Hyperventilating Decrease PCO2 Respiratory alkalosis
528
Bioavailability on graph
area under the curve
529
Inject 5% saline what changes
Hypertonic into extracellular space water from intracellular into extracellular Decrease IC vol Increase IC osm Increase EC vol Inc osm vol
530
Hypotonic saline Isotonic saline Hypertonic saline
.45% saline= hypo .9%= isotonic 3%= hypertonic
531
Explains impaired action potential in axons of people with MS
Increased axonal capacitance
532
Prostate cancer spread to by means of
Spine Prostatic venous plexus to vertebral venous plexus
533
Mechanical ventilation on - peak inspiratory alveolar - end-tidal alveolar - peak inspiratory intrapleural - end-tidal intrapleursal
Positive on all
534
Pap smear columnar cells seen where?
Cervical canal Doesn't reach endometirum
535
Histology of female parts
Labia major= SS keratinized Labia minor= SS nonkeratinized ``` Vagina= ss nonkeratinized Ectocervix= ss nonkeratinized ``` Endocervix= simple columnar Body uterus= simple columnar
536
Fluoxetine affects neurons arising from
SSRI From Raphe nucleus
537
NE is from
Locus cereuleus
538
GABA from
Nucleus accumbens
539
Dopamine from
Ventral tegmentum SNc
540
Acetylcholine from
Basal nucleus of Meynert
541
Erythematous scaly rings with central clearing From pet
Tinea corporis
542
Leukomoid vs CML
Leukomoid - high alkaline phosphate (LAP) CML - low leukocyte alkaline phospahtase
543
Strawberry hemangioma
Benign capillary hemangioma of infancty
544
Renal artery aneurysn due to
Fibromuscular dysplasia
545
Silicosis, asbestosis, coal workers lung FEV1/FVC FVC Diffusion capacity of lung for carbon monoxide
Interstitial lung diseases FEV1/FVC: normal FVC: Decreased Diffusing capacity: Decreased (short swallow breaths)
546
White plaque on side of tongue what happens if dont tx
Hairy leukoplakia EBV Progress into submucosa
547
Black escar nose
Mucor
548
Ixodes deer tick
Borrelia burgdorferi Anaplasma Babesia
549
Part of eye with least impact on vision
Iris
550
``` Fever Diarrhea Splenomegaly Muscle wasting Pancytopenia ```
Bitten sand fly Leishmania spp
551
What shows up right after a burn with a blister
Histamine
552
Phototherapy does what
Increased conversion of bilirubin to water soluble isomers
553
18 month old failure to thrive and frequent wet diapers. 3rd percentile for length and weight. Physical examination shows dehydration, decreased muscle tone, and bowing of legs. Slit lamp examination shows crystals in corneas. Hypophosphatemia, hypokalemia, nonanion gap metabolic acidosis. pH of 5, glucosuria, phorphaturia, and generalized aminoaciduria. Cuase of metabolic acidosis in this patient
Decreased sodium bicarbonate reabsorption in the proximal tubule
554
Bulimia Nervosa - potassium - chloride - bicarbonate - acidosis/ alkalosis
K - decreased Chloride - increased Bicarbonate - decreased Alkalosis
555
New born 2 weeks old, yellow skin for 7 days. At birth, lethargic and hypoglycemia. Vomits at feedings. PE shows jaundice. There is no glucosuria but reducing substances are detected in the urine. Direct bilirubin concentration is increased. An assay for what will give a diagnosis
Galactose-1-phosphate uridyltransferase
556
Normoblast Megakaryocyte
Normoblst: production of RBC Megakaryocyte: production of platelet
557
Leukotrienes do what
LTC4 LTD4 LTE4 Increase bronchial tone LBT4 - increase neutrophil chemotaxis
558
Prostacyclin do what
Decrease platelet aggregation Decrease vascular tone
559
Prostaglandins do what
Decrease vascular tone Increase uterine tone
560
Thromboxane do what
Increase platelet aggregation Increased vascular tone
561
Small cell lung cancer - secretes tx
SIADH Demeclocycline
562
Split S1. First component of sound
Closure of mitral valve
563
Cerebellar hemisphere lesion on right see deficits on
Right
564
Lateral eye movement
CN VI Pons
565
Stellate gangion stimulation
heart rate
566
Chronic renal failure - Prosphate - Ca - PTH
Phosphate increase Ca decrease Parathyroid increase
567
Crash crash Fractures and lacerations Tachypnea, tachycardia and cyanosis Diffuse consolidation of both lungs what is seen on autopsy
Hyaline membranes acute respiratory distress
568
Leukocyte differential
WBC no T cell or B cell
569
How are integral membranes anchored
Extensive hydrophobic interactions between the amino acid side chain of the protein and the membrane phospholipid tails
570
Bronze diabetes
Hemochromatosis HFE gene mutation chr 6 HLA-A3 Increased ferritin Increased iron Decreased TIBC Increased transferrin saturation
571
Microcytic anemia types
Thalassemia Anemia of chornic disease Iron deficiency Lead poisoning TAIL
572
Microcytic anemia | Asain
Alpha thalassemia
573
Most common lung cancer
adenocarcinoma
574
Disease form bed bugs
Staph aureus
575
Erectile dysfunction nerve
Cavernous n. Inferior hypogastric form pelvic splanchnic
576
Sperm missing tails
Primary ciliary dyskinesia (PCD) AR Bronchiectasis another symptoms Situs inversus
577
E coli makes many proteins how
mRNA contains sequence for many proteins
578
Scratches Vetarianian Parrot Tender regional lymphadenopathy
Bartonella henselae
579
Bartonella henselae can cause what in immunocompromised
Bacillary angiomatosis Red-purple papular skin lesions
580
Pulmonary embolism does what to pH, PaO2, PaCO2, Serum HCO3
pH increase PaO2 decrease PaCO2 decrease Serum HCO3 Normal PE occludes parenchyma resulting in increased dead space ventilation Hyperventilation --> hypocapnia and respiratory alkalosis Acute so no bicarb involvement yet
581
Fever myalgias, malaise, and progressive fatigue over 2 week period No sore throat Splenomegaly No Lad or jaundice Fails to agglutinate horse erythrocytes
Mono (normally EBV0 but since no agglutination CMV
582
Regulates glucose homeostasis during prolonged fasting by altering transcription of many key enzymes
``` Cortisol (cytoplasm) Growth hormone ( membrane-bound) ```
583
Splenomegaly on histology
Red pulp expansion
584
Additional thing to check if alkalosis
Urine Cl - checks vomiting - checks diuretic
585
Noscomal infections
Coag neg staph Staph aureus Enterococci Candida
586
Increase in use of what has increased staph noscomal infections
Intravascular catheters
587
Strongest factor for risk of completed suicide
Past suicide attempt
588
Arthritis Drug for treat causes Mouth ulcers Elevates AST ALT
Methotrexate
589
Most infections that arise after chemotherapy are from
Patients own endogenous bacterial flora
590
Tetralogy of fallot squatting does what
Ventricular septal defect Overriding aorta Increases systemic vascular resistance Increase SVR causes a higher proportion of right ventricular output into pulmonary circulation rather than into left ventricle
591
Foot drop Loss sensation on dorsum of foot Nerve compressed where
Fibular neck
592
Mycoplasma and anemia
Cross rxn of IgM antibodies to red blood cells, activate complement and cause erythrocyte lysis Cold agglutinins Hemolytic anemia IgM titers fall with infection resolution
593
Brain natriuretic peptide levels secreted from
Ventricular myocytes Due to increased ventricular wall stress
594
5 y.o to ER iwth 2 days of dark, low-volume urine and decreased energy. Had abdominal pain, fever and bloody diarrhea after eatting hamburgers 3 days ago. No edmea or rashes. Anemia, thormocytopenia and elevated BUN and creatine. See elevated waht
HUS from E.coli Shigella toxin Decease in hemoglobin and haptoglobin Increase lactate dehydrogenase and unconjugated bilirubin levels
595
Bacteria virulence factor simlar to tropomyosin and myosin. Fxn
M protein Resist phagocytosis Group A streptococcus - S pyogenes - cross rxn causes rheumatic carditis
596
What contributes to hypocalcemia in patient with chronic renal disease
Hyperphosphatemia Phosphate binds free calcium and precipitates in soft tissue Triggers fibroblast growth factor, which lowers phospahte levels by inhibiting 1 alpha hydroxylsae Decreases production of 1,25 hydroxyvitamin D
597
Decreased pulmonary capillary wedge pressure
Right sided heart failure Impaired blood flow from right ventricle to left side of heart
598
Drugs and renal failure
Acute interstital nephritis
599
Delayed puberty | Anosmia
Kallmann syndrome Failure of GnRH secreting neurons to migrate from origin in olfactory placode to normal anatomic location in hypothalamus Mutation KAL-1 gene May have cleft lip or palate
600
Antibodies to phospholipase A2 (PLA2R)
Membranous nephropathy
601
Magnesium salts and aluminum hydroxide together for antiacid
Magnesium causes diarrhea Aluminum hydroxide causes constipation Both temporarily increase the gastric pH by neutralizing hydrochloric acid relieving reflux
602
Common source of hemorrhage from penetrating gastric ulcers
Right and left gastric arteries that run along lesser curvature of stomach
603
What if taking during pregnancy can cause oligohydramnios
ACE inhibitors and ARBs
604
Pleural effusion - due to pressure changes - due to inflammation
Pressure changes - transudative - heart failure - cirrhosis - nephrotic syndrome Inflammation - Exudative - increase vascular membrane permeability (infection, malignancy, rheumatic disease) - fluid to serum ratio of protein > 0.5
605
3 week old Abdominal distension vomiting blood streaked stools Had normal stool and urine X ray shows thin curvilinear areas of lucency that parallel the bowel of the lumen
Pneumatosis intestinalis (air in the bowel wall) Necrotizing enterocolitis (NEC)
606
Man with behavioral changes Lack of focus and poor memory Involuntary jerky movements
Huntington's disease CAG trinucleotide repeats GOF mutation Abnormal protein build up causes loss of inhibitory (GABA) neurons Atrophy of caudate nucleus
607
Hiatal hernia caued by
Circumferential laxity of the phrenoesophageal membrane
608
IgA dn C3 deposition in vascular lesions of thigh
Henoch-Schonlein purpura (HSP)
609
Saddle nose and oliguria CXR?
Granulomatosis with polyangiitis (Wegeners) Large nodular densities
610
Which testicular tumor is associated with each? 1) Composed of cytotrophoblasts and syncytiotrophoblasts? 2) May present initially with gynecomastia 3) Elevated AFP 4) Elevated beta-hCG 5) Histologic appearance similar to koilocytes (cytoplasmic clearing)
1. Choriocarcinoma 2. Leydig or sertoli cell tumor 3. yolk sac tumor 4. choricocarcinoma, embryonal carcinoma 5. Seminoma
611
Which ovarian tumor matches each? 1) Estrogen secreting leading to precocious puberty 2) Produces AFP 3) Psammoma bodies 4) Testosterone secreting leading to virilization 5) Lined with fallopian tube like epithelium 6) Ovarian tumor + ascites + hydrothorax 7) Call-Exner bodies 8) Elevated beta-hCG 9) Resembles bladder epithelium
1) Granulosa theca cell tumor 2) Yolk-sac tumor 3) Serous cystadenocarcinoma 4) Sertoli-leydig cell tumor 5) Serous cystadenoma 6) Ovarian fibroma (meigs syn) 7) Granulosa cell tumors 8) Choriocarcinoma, dysgerminoma 9) Brenner tumor
612
Which embryologic structure of the heart gives rise to each of the following? 1) Ascending aorta and pulmonary trunk 2) Coronary sinus 3) Superior vena cava
1) Truncus arteriosus 2) Left horn of sinus venosus 3) Right common cardinal vein and right anterior cardinal vein
613
What is the antidote for 1) Iron 2) Lead 3) cyanide 4) Methomoglobin 5) Carbon monoxide 6) Methanol or ethylene glycol
1) Deferoxamine 2) EDTA , succimer, dimercaprol 3) Nitraites, hydroxycobalamin, thiosulfate 4) Methylene blue, vit C 5) 100% oxygen hyperbaric oxygen 6) Fomepizole, ethanol, hemodialysis
614
Wound healing after 1 week
Fibroblasts Type III collagen replaced by type I collagen
615
Exudative vs transudate - Looks like - Lab - Due to
Exudate - Cellular (cloudy) - Increased protein > 2.9 , increased LDH (vs serum) - Lymphatic obstruction, inflammation, infection, malignancy Transudate - Hypopcellular (clear) - Decrease protein < 2.5, decreased LDH vs serum - Increased hydrostatic pressure (HF, Na retention) - Decreased oncotic pressure (cirrhosis, nephrotic syndrome)
616
Hematogenous spread of carcinomas
Four Carcinomas Route Hematogenously Follicular thyroid carcinoma Choriocarcinoma Renal cell carcinoma Hepatocellular carcinoma
617
Low Vd - compartment - drug types
Intravascular Large/ charged molecules Plasma protein bound
618
High Vd - compartment - drug types
All tissues including fat Small lipophilic molecules, especially if bound to tissue protein
619
Clearance (CL)=
Rate of elimination of drug/ plasma drug concentration = Vd x Ke (elimination constant) Ke= 0.7/t(1/2) CL= (0.7 x Vd)/ t(1/2)
620
Half life (t1/2) =
0. 7 x Vd/ CL 3. 3 half lives = 90% steady state ``` % remaining 1 half life= 50% 2= 25% 3= 12.5% 4= 6.25% ```
621
Loading dose=
(Cp x Vd)/ F F= bioavailability IV F= 100% Cp= target plasma conc at steady state
622
Maintenance dose=
(Cp x CL x tau)/ F tau= dosage interval (time between doses, if not administered continuously, in hours) Maintenance dose for 1 day= 24 hrs
623
Therapeutic index=
TD50/ ED50 Median toxic dose/ median effective dose The higher the TI the safer the drug
624
Drugs with low Therapeutic index
Warning! These Drugs are Lethal Warfarin Theophylline Digoxin Lithium
625
Nicotinic ACh receptors are Muscarinic ACh receptors are
Nicotinic ACh receptors are ligand gated Na/K channels Muscarinic ACh receptors are G protein coupled receptors through second messengers
626
Alpha 1 fxn (3)
Increase vascular smooth muscle contraction Increase pupillary dilator muscle contraction (mydriasis, dilation) Increase intestinal and bladder sphincter muscle contraction
627
Alpha 2 fxn (5)
``` Decrease sympathetic (adrenergic) outflow Decrease insulin release Decrease lipolysis Increase platelet aggregation Decrease aqueous humor production ```
628
Beta 1 fxn (4)
Increase HR Increase contractility (one heart) Increase renin release Increase lipolysis
629
Beta 2 fxn ( 7)
``` Vasodilation Bronchodilation (two lungs) Increase lipolysis Increase insulin release Decrease uterine tone ** Ciliary muscle relaxation Increase aqueous humor production ```
630
M1 fxn M2 fxn M3 Fxn
M1: mediates higher cognitive functions, stimulates enteric nervous system M2: Decrease HR and contractility of atria M3: Increase exocrine gland secretions, increase gut peristalsis, increase bladder contraction, bronchoconstriction, increase pupillary sphincter muscle contraction (miosis)
631
Conotruncal abnormalities associated with failure of
Neural crest cells to migrate: Transposition of great vessels Tetralogy of fallot Persistent truncus arteriosus
632
Baby takes first breathe
Decrease resistance in pulmonary vasculature Increase pressure in left atrium
633
Preload - is what - approximated by - also measures - decreased by
Pressure at end of diastole (relaxation) when ventricles are the fullest right before contraction. Approximated by ventricular End-diastolic volume Ventricles fill from atria, atrial pressure= preload Decreased by venous vasodilators
634
Afterload - approximated by - decreased by
Approximated by Mean arterial pressure Total peripheral resistance (TPR) Decreased by arterial vasodilators
635
EDV =
Right atrial pressure
636
S1 heart sound S2 heart sound
S1= mitral and tricuspid closing S2= aortic and pulmonary closing
637
Beside maneuver 1) Inspiration 2) Hand grip 3) Valsalva, standing up 4) Squatting
1) Inspiration - increases venous return to right atrium - Increase Intensity of right heart sounds 2) Hand grip - Increase after load - Increase intensity of MR, AR, and VSD - Decrease AS murmur 3) Valsalva, standing up - Decrease preload - Decrease intensity of most murmurs - Increase intensity of hypertropic cardiomyopathy 4) Squatting - Increase venous return, increase preload, increase afterload - Increase intensity of AS, MR, VSD
638
Holosystolic murmur
Tricuspid regurg | Mitral regurg
639
Slurred curved upstroke (delta wave) between P and R
Wolff parkinson white Abnormal fast accessory conduction pathway from atria to ventricle (bundle of kent) bypassing AV node May result in SVT
640
Origination site of atrial fibrillation
Pulmonary vein ostia
641
Atrial flutter caused by
Large reentrant circuit that transverses the cavotricuspid isthmus (region of R atrial tissue between IVC and tricuspid valve annulus)
642
Atrial natriuretic peptide released due to
Increase in blood volume and atrial pressure acts as cGMP Vasodilation and Decreased Na reabsorption
643
Pulmonary capillary wedge pressure approximation of
left atrial pressure
644
Rheumatic fever - valve - tx
Early: mitral regurg Late: mitral stenosis Penicillin
645
Innumerable renal microaneurysms and spasms on arteriogram Fever, WL, Headache Abdominal pain HTN
Polyarteritis nodosa
646
Drugs that cause pulmonary fibrosis
Amiodarone Bleomycin Busulfan
647
Drugs taht cause flushing
Vancomycin Niacin Adenosine Dihydropyridine Ca channel blockers
648
Drugs that cause Torsades de pointes
``` Potassium channel blockers Na channel blockers Macrolides Haloperidol Chloroquine Protease inhibitors ```
649
Drugs that cause gingival hyperplasia
Phenytoin | Verapamil
650
GI bleeding | Buccal pigmentation
Peutz jegher syndrome
651
Tuberous Sclerosis
HAMARTOMASS ``` Hamartomas in CNS and skin Angiofibromas Mitral regurg Ash lead spots Cardiac rhabdomyoma Tuberous sclerosis autosomal dOminant Mental retardation renal Angiomyolipoma Seizures Shagreen patches ```
652
78 y.o to office with one month history of progressive dyspnea, generalized weakness, fatigue and palpitations. He reports tingling and numbness in both lower limbs. Hes not been taking care of himself since wife died. CV exam shows displaced apical impulse at 6th intercostal space, a third heart sound, and high volume carotid pulses. Pedal edema, mild hepatomegaly. Normal blood counts. What deficiency? ``` A. Ascorbic acid B. Cobalamin C. Niacin D. Pyridoxine E. Retinol F. Riboflavin G. Thiamine ```
G. Thiamine Wet beriberi - peripheral neuropathy - heart failure
653
Person dies after 5 days from heart failure due to
Free wall rupture --> Profound hypotension and shock
654
History of mitral valve prolapse. Temp of 100. Mid systolic click systolic murmur at apex. Gram positive bacteria that synthesizes dextrans from glucose. Adherence of bacteria would be where? ``` A. Circulating heparins B. Endothelial surface glycoproteins C. Fibrin platelet aggregates D. Subendothelial collagen E. Subendothelial glycosaminoglycans ```
C. Fibrin platelet aggregates Virdians streptococci. Produce dextrans which facilitate adherence to fibrin. Fibrin and platelets are deposited at sites of endothelial truma
655
What is similar in parameters between systemic and pulmonary circulation ``` A. Arterial oxygen content B. Arterial resistance C. Blood flow per minutes D. Diastolic arterial pressure E. Driving pressure for blood flow F. Mean arterial pressure ```
C. Blood flow per minute
656
Superior gluteal and inferior gluteal come off
Internal iliac also obturator comes off
657
Chronic anemia on cardiac output and venous return curves
Increase in cardiac output Somewhat increase in venous return
658
Anaplylaxis effect on cardiac otuput and venous return curves
Widespread venous and arteriolar dilation Drop in venous return (shifted down and leftward)
659
1 week old infant Discharged at 30 hrs PE harsh, holosystolic murmur at left mid to lower sternal border No murmur heard at birth
Non-cyanotic possibilities - Atrial septal defect - VSD - PDA - Coarctation of aorta Patients presents w/ VSD - loud blowing holosystolic murmur
660
What chemical substance help decrease thrombus propagation by inhibiting platelet aggregation?
Prostacyclin
661
Normal cardiac pressures 1) RA 2) RV 3) Pulmonary artery 4) Pulmonary artery wedge 5) LA 6) LV 7) Aortia
1) RA= < 5 2) RV= 25/5 3) Pulmonary artery= 25/10 4) Pulmonary artery wedge= < 12 (10) 5) LA= < 12 (10) 6) LV= 130/10 7) Aorta= 130/90
662
Atrial myxoma on histology
Amorphous extracellular matrix With scattered stellate or globular myxomas cells within abundant mucopolysaccharide (myxoid) ground substance containing chondroitin sulfate and hyaluronic acid
663
Common cardinal veins form
Superior vena cava
664
Disorder in which no finding can be found to support symptoms but recently had trauma or stress in life
Conversion disorder
665
Cachectin caused by
TNF-alpha IL-1 IL-6
666
Liver mass that is a cystic lesion Hepatomegaly - microscopic examination - complication of treatment
Echinococcus granulosus Hydatid cysts Sheep/ dog expsoure Eggshell calcifications Caution with removal --> spilling of cyst contents can cause anaphylactic shock
667
Blistering in sun | Itchy
Porphyria cutaneous tardia Uroporphyringoen decarboxylase
668
Painful genital lesion, irregular ragged borders Grey excudatecovering Enlarged inguinal LN
Chancroid`
669
Fatigue Early satiety Mucosal pallor with no scleral icterus. Lungs clear. Mild hepatomegaly. Can feel tip of spleen. Pancytopenia. Peripheral blood= immature granulocytes, nucleated erythrocytes and teardrop cells. BM aspiration is attempted but yields no marrow - disease - MOA - Secretes - Symptoms - Histopathylogical features
Primary myelofibrosis Chronic myeloproliferative disorder with clonal megakaryocytes Secrete TGF-beta --> fibroblasts to produce collagen and BM fibrosis --> Extramedullary hematopoiesis Massive splenomegaly Tear drop cells Dry tap Large islands of hematopoietic progenitor cells
670
Spleen histopathyology 1) Accumulation of macrophages with fibrillary cytoplasm 2) Diffuse neutrophilic infiltration and follicular necrosis 3) Dilated sinusoids and fibrosis nodules with hemosiderin
1) Gaucher disease - AR lysosomal storage disease - fibrils of glucocerebroside (Crumped tissue paper) 2) Acute infection of spleen Follicular necrosis= infection group A strep 3) Congestive splenomegaly due to portal HTN
671
Dermatomyositis - biopsy shows - can affect
Proximal muscle weakness Rash on eyelids Affects striated muscles and skin Oropharynx and upper esophagus Perimysial inflammatory infiltrates and atrophy involving the fibers around the periphery of muscle fascicles (perifascicular atrophy)
672
Eosinophilic esophagitis presents as
Dysphagia Epigastric pain Recurrent esophageal relfux Food impaction
673
NE leak into arm causes Tx
Alpha 1 receptor mediated vasoconstriction which can lead to tissue necrosis Tx Phentolamine (alpha receptor blocker)
674
Thyroid Fine needle aspiration biopsy Clusters of cells with large overlapping nuclei containing sparse, finely dispersed chromatin Numerous intranuclear inclusion bodies and grooves are seen
Papillary carcinoma Overlapping nuclei containing finely dispersed chromatic (empty or ground glass appearance= orphan annie eyes)
675
``` Unusually muscle movements of thumb Hypoplastic mandible Low set ears Bifid uvula Cleft palate ```
Physical features of DiGeorge syndrome Hypocalcemia
676
Heme synthesis occurs where
Mitochondria
677
New born with drug addict mother. Irritable, crying, tremors, sneezing and diarrhea Tx
Opioid withdrawal - Neonatal abstinence syndrome Morphine Methadone
678
COPD lung volumes
Air trapping and hyperinflation Higher functional residual capacity Higher reserve volume Total lung capacity increases as well but to a lesser extent High RV/ TLC ratio Diffusing capacity of lung for carbon monoxide is decreased - due to emphysematous destruction of the alveolar arterial membrane FEV1 decreased FVC decreased FEV1/FVC decreased
679
Loss of internal elastic lamina
Fibromuscular dysplasia Luminal stenosis alternating with areas of dilation Resistant HTN Stroke Ruptured aneurysm Renal artery stenosis Cerebrovascular involvment
680
Other form of downs syndrome
Trisomy 21 46, XX t(14;21) Robertsonian translocatoin
681
HTN Suppressed Renin Na? K? Bicarb
Primary aldosteronism High Na Low K Low Bicarb
682
Sudden cardiac death due to
Ventricular fibrillation | - Lack of tissue perfusion due to blockage
683
Horseshoe kidney associated iwth
Turner
684
Schistocytes associated with
HUS, TTP, DIC and AORTIC STENOSIS
685
Structure gives rise to heart structure 1) Smooth parts of left and right ventricles 2) Smooth part of right atrium 3) Trabeculated left and right atria 4) Trabeculated parts of the left and right ventricles
1) Smooth parts of left and right ventricles - Bulbus cordis 2) Smooth part of right atrium - Right horn of sinus venosus 3) Trabeculated left and right atria - Primitive atria 4) Trabeculated parts of the left and right ventricles - Primitive ventricle

AM- STEP1 (20 decks)

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