Hematology/Oncology Flashcards
(126 cards)
1
Q
What do you do next when diagnosing megaloblastic anemia by vitamin B12 deficiency?
A
Confirm the etiology before treatment (better route is intramuscular)
*Dietary absence (vegans), pregnancy, malabsorption syndrome, ileal or gastric resection, pernicious anemia
2
Q
History suggestive of pernicious anemia. How do you confirm the diagnosis?
A
- Dyspepsia, autoimmune condition (ex, diabetes type 1), elderly
- Low B12 levels
- Confirm: Serum anti-intrinsic factor antibodies and anti-parietal cell antibodies
3
Q
What is the therapy for Heparin-induced Thrombocytopenia (HIT)?
A
- Stop Heparin (Unfractionated or LMW)
- Continue anticoagulation with non-heparin medication (argatroban, fondaparinux)
*Lepirudin was discontinued
4
Q
Most common cause of sepsis in sickle cell disease
A
Streptococcus pneumoniae→despite immunization, is due to non-vaccine serotypes
5
Q
Tumors that cause approximately 75% of all malignant pleural effusions
A
Lung carcinoma, breast carcinoma and lymphoma
6
Q
What is the cause of Febrile nonhemolytic transfusion reaction and how do you avoid it?
A
- Cytokine release from leukocytes within the donor blood product
- Leukoreduction of blood product
*Most common adverse transfusion reaction
7
Q
Malignancies risks in a female patient with Lynch syndrome. Screening indication.
A
- Colorectal cancer➡colonoscopy every 1–2 years starting at 25 years
- Endometrial carcinoma➡annual endometrial biopsy beginning 30-35 years
- Ovarian cancer
*If childbearing is complete, prophylactic hysterectomy and bilateral oophorectomy is recommended at 40 or earlier
8
Q
Mechanism of jaundice in Vitamin B12 deficiency
A
Ineffective erythropoiesis▶⬆immature erythrocyte megaloblasts in bone marrow➡⬆intramedullary hemolysis➡⬆Heme▶⬆indirect bilirubin
9
Q
Most common complication of Hemophilia A treatment with recombinant factor VIII
A
Inhibitor development➡immune system recognizes factor as foreign▶antibodies ❌factor
*⬆Bleeding frequency or hemorrhage refractory to treatment, ⬆PTT
10
Q
Treatment for inhibitor development in a patient with Hemophilia A
A
- Recombinant activated factor VII
- Activating prothrombin complex concentrates
11
Q
Main manifestations of Von Hippel-Lindau disease
A
- Hemangioblastoma in the retina and cerebellum (cystic nodular pattern)
- Renal cell carcinoma (clear cell subtype, bilateral), preceded by multiple renal cysts (premalignant)
- Pheochromocytoma
*Autosomal dominant
12
Q
Hallmark of Paget’s disease and underlying breast condition
A
- Painful, itching, eczematous, and/or ulcerating rash on the nipple that spreads to the areola
- Adenocarcinoma
13
Q
How do you confirm the diagnosis of Heparin-Induced Thrombocytopenia (HIT)?
A
- Immunoassay➡⬆Titers
- Functional assay (Serotonin release assay) [Gold standard but less available]
14
Q
Risk factors for splenic infarction
A
- Hypercoagulable disorder
- Source of embolic disease (atrial fibrillation)
- Myeloproliferative neoplasm
- Hemoglobinopathy (sickle cell disease)
15
Q
Most common cause of megaloblastic anemia in chronic alcoholics
A
Folate deficiency
*Alcohol impair enterohepatic cycle and absorption of folate, may develop anemia in 5-10 wks (body stores are limited)
16
Q
Effect of brussels sprouts, broccoli and green tea on warfarin
A
↓Effect→↑Thrombosis risk NO bleeding risk
17
Q
Effect of acetaminophen on warfarin
A
↑Effect→↑Bleeding risk
18
Q
Pathophysiology mechanism of Graft versus host disease
A
Activation of donor T-cells by recognition of host HLA-antigens▶cell-mediated immune response (Type IV hypersensitivity reaction)
19
Q
Clinical presentation of chronic Graft versus host disease
A
- Sicca syndrome
- Chronic enteritis: Bloody diarrhea, Abdominal pain, Weight loss
- Hepatic dysfunction: jaundice
- Bronchiolitis obliterans: Chronic cough, Wheezing, Dyspnea
- Myasthenic symptoms
- Polymyositis
- Scleroderma-like and lichenoid skin changes
*> 100 days after transplantation
20
Q
When can you use anti-D immune globulin on Immune thrombocytopenia?
A
First-line treatment along with IVIG and corticoids in children if bleeding, and adults if bleeding or platelets≤30.000/mm3, and if Rh-positive and coombs negative→saturate Fc receptors of macrophages within the reticuloendothelial system (RES)►limiting the RES to clear platelets
*Do not give in Rh-negative patients
21
Q
Cause of hemarthrosis with normal coagulation times and platelets in a patient with a history of bariatric surgery or restricted food intake
A
Vitamin C deficiency (scurvy)
22
Q
Giant cell tumor of bone clinical presentation
A
- Progressive knee pain + X-ray with eccentric lytic lesion (“soap bubbles”)
- Benign, locally destructive neoplasm at epiphysis of long bones (young adults)
23
Q
Giant cell tumor of bone association and prognosis
A
- Associated with paget disease of bone
- It is benign but might have malignant transformation and pulmonary metastasis
24
Q
Osteoid osteoma etiology and clinical presentation
A
- Benign bone tumor prostaglandin-producer▶Nocturnal pain relieved by NSAIDs
- X-ray: Small, round lucency
25
Clinical presentation of acute hemolytic transfusion reaction
- Onset in minutes to 24 hours of transfusion
- Fever, chills, hypotension
- Renal tubular cell injury (may progress acute renal failure)▶Hemoglobinuria (dark urine), flank pain
- Intravascular Hemolysis➡⬆LDH, ⬆Indirect bilirubin
- DIC➡oozing intravenous site
26
Diagnosis test for acute hemolytic transfusion reaction
Direct coombs test➡antibody-coated RBCs
27
Primary dose-limiting side effect of first-line treatment for Sickle cell anemia with recurrent vaso-occlusive crisis
Myelosuppression (neutropenia, anemia, thrombocytopenia)
*Hydroxyurea➡⬆fetal hemoglobin➡⬇sickled hb➡⬇polymerization of RBC▶⬇vaso-occlusion episodes
28
Most common site of colon cancer metastasis
Liver
29
Musculoskeletal presentation of hereditary hemochromatosis
Arthralgia, arthropathy, chondrocalcinosis (Pseudogout)
30
How you may differentiate small cell carcinoma vs squamous cell carcinoma of the lung?
Paraneoplastic syndromes:
- Small CC: ⬆ACTH (Cushing sx), ⬆ADH (SIADH), ⬆Antibodies against presynaptic Ca+2 channels (Lambert-Eaton myasthenic sx)
- Squamous CC: ⬆PTHrP (hypercalcemia)
* Sca++mous
31
Best treatment for cancer-related anorexia/cachexia syndrome (CACS)
Progesterone analogs (megestrol acetate medroxyprogesterone acetate)>corticosteroids
32
Which test you should order in an adult patient with isolated thrombocytopenia (Immune Thrombocytopenic Purpura)?
HIV and Hepatitis C➡most common secondary causes
*Initial presentation HIV up to 5-10%
33
Pathophysiology of Fanconi anemia
Autosomal recessive DNA repair defect, Bone-marrow failure
34
Clinical findings of Fanconi anemia
- Short stature
- Hypo or hyper-pigmented macula on trunk
- Genitourinary malformations
- Absence or hypoplastic thumb
- Polydactyly, flat thenar eminence
*Most common cause of congenital aplastic anemia
35
Two most common inherited thrombophilia in the caucasian population
1. Factor V Leiden (Activated Protein C resistance)
| 2. Prothrombin mutation (⬆Prothrombin levels)
36
When do you study or test for hereditary thrombophilias?
- Young age (<45) with first time unprovoked DVT/PE
- Recurrent DVT/PE
- Unusual sited of thrombi (cerebral, mesentery, portal veins)
37
Treatment to reverse Warfarin toxicity bleeding
Prothrombin complex concentrate➡Vitamin K dependent coagulation factors (II, VII, IX, X, Protein C and S)
* Room temperature
* No transmit disease
* No excess volume
38
Hemoglobin electrophoresis pattern of Beta-thalassemia major
- HbA2 ⬆⬆ (2 alpha + 2 delta)
- HbF ⬆⬆ (2 alpha + 2 gamma)
* Mutation in both beta-globin genes
* HbS absent, HbA absent
39
Treatment of Beta-thalassemia major
Transfusion-dependent➡⬆Risk of iron overload➡Iron chelation therapy▶avoid damage on liver, kidneys, endocrine glands
40
Findings on laryngoscopy of laryngeal squamous cell carcinoma in a patient with hoarseness
Fungating, irregular white/red mass on vocal cord with/without blood crusting (ulcer)
41
When do you suspect carcinoid syndrome?
Intermittent diarrhea + episodic flushing (85%) + cutaneous telangiectasias + bronchoespasm (wheezing) + cardiac abnormalities (right heart HF, tricuspid regurgitation)
42
Best initial diagnostic test for carcinoid syndrome
Urinary 5-hydroxyindolacetic acid (5-HIAA)
*Degradation product of Serotonin
43
Treatment for carcinoid syndrome
Octreotide
44
Most common benign cystic mass/tumor in the middle mediastinum
Bronchogenic cyst
45
Electrolyte abnormalities in Tumor lysis syndrome and their consequences
- ↑Phosphorus
- ↑Potassium→cardiac arrhythmia
- ↑Uric acid→uric acid stones + AKI
- ↓Calcium→calcium-phosphate stones + AKI
*Hypocalcemia is due to Hyperphosphatemia→↑calcium-phosphate precipitation (binding in the renal tubules)
46
What is the Her2/neu test and why is it done in breast cancer?
- Abnormal estrogen receptor
| - Positive➡anti-Her2/neu antibodies➡Trastuzumab▶⬇risk of recurrence and ⬆survival
47
Which is the hormonal manipulation or treatment for breast cancer? when are they indicated?
- Tamoxifen or Raloxifene: premenopausal
- Aromatase inhibitors (anastrozole, letrozole, exemestane): postmenopausal, slight superior in efficacy
- All Estrogen or Progesterone Receptor patients: prevent recurrences
48
Most important adverse effects of tamoxifen and aromatase inhibitors
- Tamoxifen➡Endometrial cancer and clots
| - Aromatase inhibitor➡Osteoporosis
49
Available hormonal therapy in prostate cancer and for what is it used?
- Flutamide, GNRH agonists, ketoconazole, orchiectomy➡help control the size and progression of metastases
- Do not prevent recurrences; Do shrink lesions
50
Difference in the treatment of seminoma and non-seminoma testicular cancer
- Seminomas: sensitive to chemotherapy and radiation
| - Non-seminomas: sensitive to chemotherapy
51
Contraindication of 5HT inhibitors for chemotherapy-induced nausea
- Ondansetron, granisetron, palonosetron, dolasetron: Best initial therapy
- QT prolongation on EKG
52
Biomarkers for testicular cancer
- Alpha-fetoprotein: only for nonseminomatous cancers
| - HCG: all testicular cancers
53
What is the Abiraterone and for what do you use it?
- Inhibitor of 17-hydroxylase➡❌synthesis of all androgens in the body (including adrenal)
- ⬇Progression of metastatic prostate cancer and risk of death by 30%
54
What is Pseudothrombocytopenia and its cause?
- Laboratory error due to platelet aggregation in vitro
- Incompletely mixed blood samples or serum antibodies to ethylenediaminetetraacetic acid (EDTA) (anticoagulant for hematologic testing)
55
How may you confirm Pseudothrombocytopenia?
- Peripheral blood smear➡large clumps of platelets
| - Draw blood sample on non-EDTA anticoagulant tube (heparin, sodium citrate)➡normalizes platelet count
56
Most common infections resulting from immunosuppression by solid organ transplantation
Pneumocystis pneumonia and Cytomegalovirus
57
Clinical presentation and findings of Leydig cell tumor
- Testicular mass
- ⬆Estrogen➡gynecomastia, loss libido, erectile dysfunction
- ⬆Testosterone➡acne, hirsutism
*Leydig cells➡Estrogen and testosterone source
58
Expected findings of sexual hormones measurements on Leydig cell tumor
- DO NOT produce B-hcg or AFP
| - ⬆Estrogen and Testosterone➡⬇⬇FSH and LH
59
Which is the only microcytic anemia that has high reticulocytes?
α-Thalassemia major or Hemoglobin H disease
60
Finding on peripheral blood smear that suggests megaloblastic anemia, what is the most probable etiology?
Hypersegmented neutrophils➡only B12 and folate deficiency, and antimetabolite medications
*Many factors can cause macrocytic anemia, but not all hypersegmented neutrophils (Ex, Alcohol)
61
Which tests may help to differentiate between B12 and folate deficiency? Why are they important when evaluating megaloblastic anemia?
- B12 deficiency: ⬆Homocysteine, ⬆Methylmalonic acid (MMA)
- Folate deficiency: ⬆Homocysteine, Normal MMA
*Remember! Both ⬆homocysteine; and in chronic or inflammatory (trauma, infection, cancer) conditions you might get normal B12 levels (⬆transcobalamin - acute phase reactant)➡get homocysteine and MMA even with normal B12 and folate levels if you have clinical suspicion
62
Complication of B12 or folate replacement, why does it happen?
Extremely rapid cell production in the bone marrow packages up all the potassium➡Hypokalemia
63
Why pancreatic insufficiency may cause megaloblastic anemia?
Pancreatic enzymes are needed to absorb B12➡remove B12 from the R-protein (carrier proteins)▶binds with intrinsic factor
64
Why alcohol can cause sideroblastic anemia?
Suppressive effect on the bone marrow
*May cause macrocytic anemia as well
65
How do you suspect an aplastic crisis in sickle cell disease? Which is the etiology?
Parvovirus B19➡First clue is a sudden drop in reticulocyte count
* Almost always ⬆⬆Reticulocyte count in SCD
66
Most accurate test for hereditary spherocytosis
Eosin-5 maleimide flow cytometry and acidified glycerol lysis test
67
Most accurate test for warm antibody autoimmune hemolytic anemia
Direct Coombs test➡detects IgG (Warm) antibody on the SURFACE of the red blood cells
68
Best initial treatment for warm antibody autoimmune hemolytic anemia. Second-line treatments if persist or more severe hemolysis.
- Best initial: Glucocorticoids (Prednisone)
- Second line:
Rituximab (usually with prednisone as part of first-line)
Intravenous immunoglobulin (IVIg)
Azathioprine
Cyclophosphamide
Cyclosporine
69
Most accurate test for cold agglutinin disease (cold antibody autoimmune hemolytic anemia)
Cold agglutinin (IgM) titer
*Direct coombs is positive only for complement
70
Treatment for recurrent episodes of warm antibody autoimmune hemolytic anemia
Splenectomy
71
Treatment for cold agglutinin disease
- Keep the patient warm
- Plasmapheresis
- Rituximab
*Steroids and splenectomy do NOT work
72
Best initial and most accurate test for Glucose 6 phosphate dehydrogenase deficiency
- Peripheral blood smear➡Heinz bodies and bite cells
| - Most accurate➡G6PD levels 1-2 months after an acute episode of hemolysis (is normal during or right after an episode)
73
Most common cause of death or life-threatening complication of paroxysmal nocturnal hemoglobinuria
Thrombosis➡most common hepatic and mesenteric veins
*Secondary causes: infection due to severe neutropenia and hemorrhage due to severe thrombocytopenia
74
Hematologic conditions associated with paroxysmal nocturnal hemoglobinuria
- Previous history of (or may cause) Aplastic anemia or bone marrow failure
- May cause Acute myeloid leukemia, myelofibrosis
75
Classical features of Paroxysmal nocturnal hemoglobinuria clinical presentation
- Episodic dark urine
- Iron deficiency anemia
Triad:
- Pancytopenia
- Hemolysis
- Venous thrombosis (severe abdominal pain)
76
Most accurate test for Paroxysmal nocturnal hemoglobinuria
CD55/CD59 absence via flow cytometry
77
Best initial treatment for paroxysmal nocturnal hemoglobinuria
Prednisone for hemolysis
78
Treatment for hemolysis and thrombosis in paroxysmal nocturnal hemoglobinuria
Eculizumab➡❌C5 in the complement pathway▶⬇Red blood cell destruction
79
Most accurate test for polycythemia vera
JAK2 mutation
80
Best therapy for polycythemia vera when hydroxyurea fails
Ruxolitinib➡inhibitor of JAK2
81
Most accurate test for acute leukemia
Flow cytometry will distinguish the different subtypes of acute leukemia
82
Indications of exchange transfusion in sickle cell disease
Severe vasoocclusive crisis:
- Acute chest syndrome (pulmonary infarction, pneumonia)
- Priapism
- Stroke
- Retinal infarction
83
Treatments options for aplastic anemia besides supportive management
- Allogenic bone marrow transplantation (BMT)➡young patients with a matched donor
- Severe cases, no stem cell donor, or patients >50 yrs➡immunosuppress:
* Cyclosporine➡❌T cells (Tacrolimus is alternative)
* Antithymocyte globulin (ATG) and eltrombopag (prevent autoimmune marrow destruction)
* Alemtuzumab➡anti-CD52▶❌T cells
84
Treatment options for Myelofibrosis
- Thalidomide, Lenalidomide➡TNF inhibitors▶⬆bone marrow production
- <55 years➡attempt allogeneic bone marrow
transplantation
- Ruxolitinib➡❌JAK2▶suppresses myelofibrosis
85
Which treatment you should add to M3 type acute myelogenous leukemia and why?
All-trans Retinoic Acid (ATRA)➡induces differentiation of promyelocytes
*t(15;17)➡PML gene; Retinoic acid receptor α▶Abnormal receptor
86
What is a leukostasis reaction?
- May happen in AML or ALL
Blasts occluding the microcirculation➡pulmonary edema, CNS symptoms, ischemic injury, DIC
*⬆⬆WBC (>150,000/mm3 in AML, > 400,000/mm3 in ALL)➡⬆⬆Risk
87
Treatment for leukostasis reaction
1. Leukapheresis
| 2. Hydroxyurea
88
Best initial therapy for chronic myelogenous leukemia
Tyrosine kinase inhibitors: Imatinib, Dasatinib, Nilotinib
*t(9:22)➡c-abl;bcr➡P210 protein▶tyrosine kinase activity
89
Most effective cure for chronic myelogenous leukemia
Bone marrow transplantation
90
Clinical presentation of acute leukemias
Bone marrow replaced by leukemia cells▶signs of pancytopenia (AML, ALL):
- Anemia➡pallor, fatigue
- ↓mature, ineffective, immature WBCs➡infections
- Thrombocytopenia➡petechiae, purpura, bleeding
- DIC most common in M3 (APL)
- Medullary expansion into the periosteum➡bone pain (ALL)
- Hepatosplenomegaly
91
Most distinct laboratory anomaly in myelodysplastic syndrome. Which others you may find?
Pelger-Huet cells➡bilobed nucleus in neutrophils ("aviator glasses")
* Also:
- Ringed sideroblasts
- Nuclear budding
- "Pawn ball" megakaryocytes
92
Treatment for myelodysplastic syndrome
- Transfusion➡support with blood products as needed
- Erythropoietin➡20% response
- Azacitidine or decitabine➡❌DNA methyltransferase➡hypomethylation of DNA (low dose), direct cytotoxicity to abnormal hematopoietic cells (high dose)
- Lenalidomide➡for patients 5q deletion➡⬇transfusion dependence
- Bone marrow transplant <50 years
93
Which ultimate complication do acute lymphoblastic leukemia and chronic lymphocytic leukemia have in common?
Transformation to Lymphoma
- ALL➡T-cell linage▶Lymphoblastic lymphoma
- CLL➡Ritcher phenomenon or transformation (5% of patients)▶Small lymphocytic lymphoma (SLL)
94
Which findings might suggest chronic lymphocytic leukemia?
- Smudge cells
- Hypogammaglobulinemia
- AIHA or autoimmune thrombocytopenia
- Lymphadenopathy
- Infections
*SHALymfect
95
Treatment for CLL
Depends on disease stage, usually III and IV
- Fludarabine➡purine analog▶interfere with ribonucleotide reductase and DNA polymerase➡❌DNA synthesis
- Cyclophosphamide➡Refractory cases or combination
- Rituximab
- Alemtuzumab➡anti-CD52 (when fludarabine fails)
- Ibrutinib➡Bruton's tyrosine kinase inhibitor
96
Treatment for Non-Hodgkin lymphoma
- Stage I/II➡Local radiation + small dose/course chemotherapy
- Stage III/IV➡CHOP + Rituximab (R CHOP)
*CHOP: Cyclophosphamide, Doxorubicin (hydroxydaunorubicin), Vincristine (oncovin), Prednisone
97
Treatment for Hodgkin lymphoma
- Stage I/II➡Local radiation + small course/dose chemotherapy
- Stage III/IV➡ABVD chemotherapy
*ABVD: Adriamycin (doxorubicin), Bleomycin, Vinblastine, Dacarbazine
98
Most common presentation of multiple myeloma. Why does it happen?
- Bone pain from pathologic fracture
| - ⬆Osteoclast activating factor (OAF)➡lytic lesions➡hypercalcemia
99
Most common causes of death in multiple myeloma
Renal failure and Infection
100
How is the anion gap in multiple myeloma and why?
⬆IgG (Cationic)➡⬆⬆Cl-, HCO3- (anions)➡⬇Anion gap
101
Most accurate test for multiple myeloma
Bone marrow biopsy➡>10% plasma cells
102
How do you expect to find the plasma protein levels in multiple myeloma?
⬆Total protein with normal albumin
103
Most common cause of IgG/A (M protein/spike) spikes on serum protein electrophoresis (SPEP). Which other causes you may think?
1. Monoclonal gammopathy of unknown origin (MGUS)
2. CLL, Lymphoma, Waldeström macroglobulinemia, amyloidosis
*Evaluate with bone marrow biopsy to exclude myeloma
104
Best initial therapy for multiple myeloma
Dexamethasone + Lenalidomide or Bortezomib, or both
105
Most effective therapy for multiple myeloma
If under 70, autologous bone marrow transplant with stem cell support
106
Clinical presentation of Waldeström Macroglobulinemia
* Hyperviscosity syndrome
- Neurological deficits➡headache, vertigo, seizures, sensorimotor peripheral neuropathy, coma
- Retinopathy➡Visual changes
- Mucosal bleeding
* Lethargy, weight loss, Raynaud phenomenon (cryoglobulinemia), engorged blood vessels on eyes ("sausage-link": dilated, segmented, tortuous)
107
Most accurate test for Waldeström Macroglobulinemia and its finding
Bone marrow biopsy➡Abnormal plasma cells with Dutcher bodies▶PAS⊕ IgM deposits around the nucleus
108
Best initial therapy for Waldeström Macrobulinemia
Plasmapheresis➡removes excess IgM➡⬇viscosity
109
Long term treatment for Waldeström Macrobulinemia
- Rituximab or prednisone + cyclophosphamide
| - Bortezomib or Lenalidomide
110
Best initial test for Von Willebrand disease
- ⬆Bleeding time in all vWD types, ⬆PTT (as in hemophilia, by ⬇factor VIII), normal PT and platelets
- ⬇vWF antigen may be present
111
Most accurate tests for Von Willebrand disease
Ristocetin cofactor assay➡⬇Agglutination
*Measures the capacity of vWF to agglutinate platelets and detects vWF dysfunction
112
Best initial therapy for Von Willebrand disease and Hemophilia if moderate or mild severity
Desmopressin➡releases subendothelial stores of vWF and Factor VIIIa
*Factor level >5% of normal (mild) or 1–5% of normal (moderate)
113
What do the hemophilias have in common regarding test results?
PT is normal, prolonged PTT➡Mixing studies with normal plasma will correct PTT (Best initial test)
114
How do you confirm Heparin-induced thrombocytopenia?
ELISA for platelet factor 4 (PF4) antibodies
115
Why Acute promyelocytic leukemia is considered an emergency?
⬆⬆Risk of pulmonary/cerebrovascular hemorrhage or blood-clotting problems from tumor-induced consumptive coagulopathy
*➕Tissue factor (DIC) + ⬆Plasmin (primary hyperfibrinolysis)
116
Treatment for acute lymphoblastic leukemia M3 (APL)
Prompt treatment with All-trans retinoic acid (ATRA)➡⬆differentiation of promyelocytes▶⬇bleeding risk
*t(15;17)➡fusion PML gene + retinoic acid receptor alpha gene
117
Clinical presentation of HUS and TTP. How you may differentiate them?
- Microangiopathic hemolytic anemia (schistocytes, helmet cells, fragmented red blood cells)
- Renal insufficiency
- Thrombocytopenia
* TTP
- Neurological symptoms➡delirium, seizure, stroke, ↓consciousness, ↓vision
- Fever
* HUS
- Hemorrhagic diarrhea preceding the syndrome (eating undercooked contaminated meat)
118
Treatment of HUS and TTP
- HUS from E. coli will resolve spontaneously
- Plasmapheresis or Fresh frozen plasma for TTP and severe HUS
- Steroids help in TTP, but NOT in HUS
119
What is Atypical HUS and its treatment?
- HUS does not arise from infection; complement erroneously attacks red blood cells
- Eculizumab➡❌complement (C5a)
120
Clinical presentation of acute Graft versus host disease
- Maculopapular rash (palms, soles, face, generalized; painful, pruritic, confluent, Steven-Johnson syndrome-like)
- Gastrointestinal: Profuse diarrhea (mucoid or watery, green or bloody), crampy abdominal pain, nausea, vomiting
- Liver inflammation (damage biliary tract epithelium)➡⬆Bilirubin, AP, transaminases; Hepatosplenomegaly
- In rare cases: lagophthalmos, hemorrhagic conjunctivitis, conjunctival pseudomembrane formation
*Within 100 days after hematopoietic stem cell transplantation
121
Pathophysiology of acute graft versus host disease
Donor T-cells (cytotoxic) identify antigens on host epithelial cells as foreign➡strong proinflammatory response
122
Most accurate test for porphyria cutanea tarda. What is the cause?
- ⬆Uroporphyrin in a 24-hour urine collection
| - Deficiency of Uroporphyrin decarboxylase activity
123
Management of malignant pericardial effusion
1. Acute drainage➡Pericardiocentesis
2. Prevention of reaccumulation➡Pericardial window* or prolonged pericardial catheter drainage
Pericardiectomy to allow pericardial fluid to drain into pleural or peritoneal cavity
124
What would be the most likely cause of kidney injury in an adult patient with hypercalcemia (progressive fatigue, muscle weakness), weight loss, and normocytic anemia?
Multiple myeloma
- Monoclonal light chains clog renal tubules▶Myeloma cast nephropathy➡Renal tubular damage (normal urinalysis)
- Amyloidosis and monoclonal immunoglobulin deposition disease➡glomerular injury and nephrotic syndrome (hematuria and proteinuria in urinalysis)
125
Why does hydroxyurea cause macrocytic anemia?
Inhibits ribonucleoside reductase (important for DNA synthesis and repair)➡inhibits erythrocyte nuclear maturation and increases nuclear/cytoplasmic ratio➡⬆MCV
126
Most likely cause of a patient with hearing loss and tinnitus after starting platinum-based chemotherapy
- Medication side effect
- Ototoxicity by cisplatin or carboplatin➡Irreversible, bilateral, high-frequency sensorineural hearing loss, tinnitus and/or imbalance
*Neurotoxic effects as well - peripheral neuropathy, CNS toxicity
