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First Aid Yellow > Heme > Flashcards

Heme Flashcards

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1
Q

No color in the iris, bircornuate uterus, mental disability

A 
Wilm's Tumor
WAGR
Wilms
Aniridia
Genitourinary malformation
Retardation
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2
Q

What is responsible for RBC concavity and flexibility

A

Spectrin

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3
Q

Varying sizes of RBCs

A

Anisocytosis

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4
Q

Vary shapes of RBC

A

Poikilocytosis

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5
Q

Immature RBCs

A

Reticulocytes

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6
Q

Deficiency of Factor VIII

A

Hemophilia A

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7
Q

Deficiency of Factor IX

A

Hemophilia B

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8
Q

Rate Limiting Step in Coag Cascade

A

X to Xa

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9
Q

PT measures which path

A

Extrinsic Path

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10
Q

PTT measures which path

A

Intrinsic Path

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11
Q

Other paths XIIa works on

A

Converts Prekallikrein to Kallikren (converts plasmingogen to plasmin) (converts HMWK to bradykinin)

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12
Q

Women with history of recurrent pregnancy loss and history of DVTs

A

Factor V Leiden

-mutant factor V resistant to degradation by protein C

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13
Q

THrombotic skin necrosis with hemorrhage after adminstration of warfarn

A

Protein C or S deficiency

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14
Q

G20290A mutation

A

Prothrombin gene mutation

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15
Q

Anticoag you follow using PTT

A

Heparin

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16
Q

cofactor for activation of antithrombin

A

Heparin

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17
Q

Antidote for Heparin OD

A

Protamine Sulfate

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18
Q

LMWH work more on facotr

A

Xa

  • longer halflife
  • can be adminitored subcutaneously
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19
Q

Why no monitoring of LMWH

A

Dosage is based on body weight

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20
Q

What cuases Heparin-induced Thrombocytopenia

A

Development of IgG antibodies against heparin-bound platelet factor 4 (PF4) –> Ab-hepatrin PF4 complex activates plateles –> thrombosis and thrombocytopenia

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21
Q

Treatment for HIT?

A

Direct thrombin inhibitors

-Bivalirudin (you were so RUDe to cause a HIT)

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22
Q

inhibits epoxide reductase

A

Warfarin

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23
Q

Monitored using PT

A

Warfarin

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24
Q

MOA of Warfarin

A

Intererferes with gamma-carboxylation of vit K-dept clotting factors (10,9,7,2) and proteins C & S
-blocks C and S first why you need to start with heparin as well

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25
Anticoag you can give during pregnancy
Heparin
26
Teratogenic affects of Warfarin
Bone and cartilate problems, nasal hypoplasia
27
OD of Warfarin
Vit K, fresh frozen plasma
28
Apixaban, Rivaroxaban
Xa inhibitors direct
29
Aids in the coversion of plasminogen to plasmin (medication)
Thrombolytics (tPA, rPA, streptokinase, tenecteplase)
30
Nephritis, deafness, cataracts
Alport Syndrome
31
IF: granular pattern of immune complex deposition, LM: hypercellular glomeruli
PSGN
32
Deficiency of Aldolase B
Fructose Intolerance
33
Deficiency of Cystathionine Synthase
Homocystinuria
34
Galactose-1-phosphate uridyltransferase def
Intellectual disability, HSM, cataracts | Classic Galactosemia
35
Deficiency of Tyrosinase
Albinism
36
What do you administer Anti-D IgG (RhoGAM) for?
Rh negative women during the third trimester to prevent maternal anti-D IgG production to avoid erthyroblastosis fetalis
37
Anti-Rh-D
Erythroblastosis Fetalis
38
RBC form in lead poisoning
microcytic anemia with basophilic stippling
39
RBC form in bone marrow infiltration (myelofibrosis)
Teadrop cell | Dacrocyte
40
RBC form G6PD def
Heinz bodies bite cells (Degamyte) | -because of that oxidized Hg, someone dug in that RBC with all their might (and by someone I mean MP)
41
Burr cell
Echinocyte (more evenly spaced and even length spurs compared to acanthocytes)
42
RBC form End-stage renal disease
Burr cell (Echinocyte)
43
RBC form Pyruvate Kinase deficiency
Burr cell (echinocyte)
44
Pencil shaped RBCs
Elliptocyte - Hereditary elliptocytosis - mutation in RBC membrane protein (spectrin)
45
RBC form in Disorders of heme synthesis
Ringed sideroblasts | -Sideroblastic anemia
46
RBC form in DIC
Schistocytes
47
RBC form in TTP/HUS
Schistocytes
48
Target cells are seen in which path?
``` HALT said the hunter to the target H: HbC disease A: Asplenia L: Liver disease T: Thalassemia ```
49
RBC form seen in patients with functional hypospelnia or asplenia
Howell-Jolly bodies | -basophilic nuclear remnants found in RBCs (normally removed by splenic MP)
50
Causes of Heinz bodies in G6PD
``` Spleen Purges Nasty Inclusions From Damaged Cells Sulfonamids Primaquine Nitrofurantoin Isoniazid Fava beans Dapsone Chloroquine TB & malaria drugs, fava beans and sulfonamides ```
51
Fetal erythropoiesis
York sac Liver Spleen Bone marrow
52
Lead poisoning inhibits which enzymes
Ferrochelatase and ALA dehydratase
53
Treatment for Lead poisoning
Succimer and EDTA
54
Symptoms of LEAD poisoning
``` LEAD Lead Lines on gums and on long bones Encephalopathy and Erythrocyte Basophilic stippling Abdominal colic and sideroblastic Anemia Drops: write and foot drops ```
55
What inhibits gamma-aminolevuliinic acid synthase?
Glucose and heme
56
What cofactor is required for gamma-aminolevulinic acid synthase?
B6
57
Defective Prophobilinogen deaminase
Acute intermittent prophyria
58
dark reddish urine, colicky abdominal pain, psychological disturbances, polyneuropathy
Acute intermittent porphyria
59
Treatment of Acute intermittent porphyria
Glucose and heme (blocks ALA anythase)
60
Symptoms of acute intermittent porphyria
``` 5 P's Painful abdomen Port wine-colored urine Polyneuropathy Psychological disturbances Precipitated by drugs, alcohol, starvation ```
61
Build up of porphobilinogen, gama-ALA, coprophiobilinogen
Acute intermittent porphyria
62
Blistering cutaneous photosensitivity, excessive hair growth, facial hyperpigmentation, increased LFTs, tea-colored urine
Porphyria Cutanea Tarda | "werewolves"
63
Defective Uropophyrinogen Decarboxylase
Porphyria Cutanea Tarda
64
Tumors that release EPO
``` Potentially Really High Hematocrit P: Pheochromocytoma R: Renal Cell Carcinoma H: Hepatocellular Carcinoma H: Hemangioma ```
65
Relative erythrocytosis
Normal number of RBCs and decreased plasma volume
66
Mutation in Sickle Cell Hemoglobin
Glutamate to Valine in Beta Chain
67
Mutation in Hemoglobin C disease
Glutamate to Lysine in Beta Chain
68
Medication inhibiting alcohol dehydrogenase
Fomepizole
69
Uses for Fomepizole
Methylene and ethylene glycol poisoining
70
50 year old patient with iron deficiency anemia check for what
Colon cancer
71
microcytosis and hypochromasia with GI bleeding
Iron deficiency
72
Iron deficiency anemia, esophageal webs, dysphagia
Plummer Vinson Syndrome | -Glossitis
73
Fatigue, light conjunctiva, pica, spoon nails
Iron deficiency anemia
74
HbH
four beta-globin forms | -In 3 allele deletion alpha-thalassem;ia
75
Causes hydropsfetalis
4 allele deletion in alpha thalassemia
76
Hb Barts
4 gamma globin forms - incompatible with life - due to 4 allele deletion in alpha thalassemia
77
Alpha thalassemia more common in what population
Asians and Africans
78
Beta thalassemia more common in what population
Mediterranean
79
Increases HbA2
Beta-thalassemia minor | -beta chain underproducted
80
microcytic, hypochromic anemia withtarget cells and increased anisopoikilocytosis
Beta-thalassemia major
81
"crew cut" on skull x ray and "chip munk" facies
Beta-thalassemia major
82
What is important to monitor with beta-thalassemia
Iron levels b/c contast blood transfusions
83
Defect in delta-ALA synthase gene
Sideroblastic anemia
84
Treatment for sideroblastic anemia
B6 (pyridoxine)
85
Test to diagnose Beta-thalassemia minor
Hg electrophoresis (look for increased A2)
86
Blood iron tests in anemia of chronic disease
Decreased iron, decreased TIBC (not main concern of body to increase iron), increased ferittin, normal % trasnferrin saturation
87
Iron studies in sideroblastic anemia
increasaed iron, decreased TIBC, increased ferritin, normal or increased % transferrin saturation -impaired heme synthesis so extra iron is lying around
88
RBC macrocytosis with hypersegmented NP and glossitis
Megaloblastic anemia - Folate def - B12 def - Orotic aciduria - Diamond-Blackfan anemia
89
Cause of megaloblastic anemia (general)
Impaired DNA synthesis --> maturation of nucleus is delayed to rest of RBC maturation, causing RBCs to expand
90
Increased homocystiene, normal methylmalonic acid, no neurological symptoms, macrocytic anemia
Folate deficiency
91
Methotrexate and Trimethoprim cause what type of anemia
Folate deficiency Macrocytic megaloblastic anemia
92
Anemia in vegans
B12 deficiency macrocytic megaloblastic anemia
93
Increased homocystiene, increased methylmalonic acid, neurological symptoms, macrocytic anemia
B12 deficiency megaloblastic macrocytic anemia | -Methylmalonic acid: impairs myelin synthesis
94
Macrocytic anemia, failure to thrive, developmental delay, no response to folate and B12
Orotic aciduria megaloblastic macrocytic anemia | -Defect in UMP synthase
95
Treatment for Orotic aciduria
Uridine monophosphate
96
Macrocytic anemia rapid-onset within 1st year of life
Diamond-Blackfan anemia | -intrinsic defect in erythroid progenitor cells
97
RBC macrocytosis without hypersegmented neutrophils
Nonmegaloblastic anemia | -causes: alcholism, and liver disease
98
MOA of anemia of chronic disease
Inflammation --> increase in hepcidin (binds ferroportin on MP and intestinal mucosal cels inhibiting iron transport) --> decreased release of iron from MP and decreased iron absorption from gut
99
Treatment of anemia of chronic disease
EPO
100
normocytic anemia, increased EPO, panyctopenia
Aplastic anemia (nonhemolytic, normocytic anemia)
101
Causes of aplastic anemia
- radiation - benzene and cancer drugs - Parovirus B19, EBV, HIV, hepatitis - Fanoconi anemia - idiopathic
102
DNA repair defect causing bone marrow failure
Fanconi anemia
103
normocytic anemia, increased indidence of tumors, cafe au lait spots, thumb and arm defects
Fanconi anemia
104
Treatment of aplastic anemia
GM-CSF bone marrow stimulation
105
Rate limiting enzyme of beta-oxidation of FA
Carnitine acyltransferase I
106
Result of glycolytic enzyme deficiency
Hemolytic anemia
107
Def in pyruvate dehydrogenase
Neurological defects
108
Defect in akyrin and specrin
Hereditary Spherocytosis
109
Causes an aplastic crisis with Parvovirus B10
- Hereditary Spherocytosis | - Sickle cell anemia
110
Positive osmotic fragility test
Hereditary spherocytosis (normocytic hemolytic anemia)
111
Anemiawith increased MCHC and increased RWD
Hereditary spherocytosis (normocytic hemolytic anemia)
112
G6PD causes what kind of hemolysis
Extravascular and Intravascular
113
MOA of Pyruvate Kinase deficiency
- Auto rec | - Defect in pyrovute kinase (RBCs cannot complete glycolysis) -> decreased ATP -> rigid RBCs -> extravascular hemolysis
114
Hemolytic anemia in a newborn
Pyruvate Kinase def (normocytic hemolytic anemia)
115
Blood smear shows hemoglobin crystals inside RBCs and target cells
HbC disease (normocytic hemolytic anemia)
116
MOA of Paroxysmal Nocturnal Hemoglobinuria
Increased completement mediated RBC lysis (impaired synthesis of GPI anchor for decay-accelerating factor that protects RBC membrane from complement) CD55/59 negative
117
CD55/CD59 negative
Paroxysmal noctural Hemoglobinuria (normocytic hemolytic anemia)
118
Eculizumab treats what
Paroxysmal nocturnal hemoglobinuria (normocytic hemolytic anemia)
119
Positive Ham's test
Paroxysmal nocturnal hemoglobinuria (normocytic hemolytic anemia)
120
Precipitates sickling of cells in SCD
low oxygen, high altitude, acidosis
121
Painful crises that occur inSCD and why
- Vaso-occlusive - dactylitis - priapism - acute chest syndrome - avascular necrosis - stroke
122
Treatment for SCD
Hydroxyurea (increases HgF)
123
RBCs are damaged when passing through obstructed or narrowed vessel lumina
Microangiopathic anemia (normocytic hemolytic anemia)
124
RBCs damaged from prosthetic heart valves and aortic stenosis
Macroangiopathic anemia (normocytic hemolytic anemia)
125
Coombs +, anemia with SLE, increased IgG
Autoimmune hemolytic anemia | -Warm
126
Coombs +,anemia triggered by cold, and Myoplasma pneumonia, blue fingers and toes from cold
Autoimmune hemolytic anemia | -Cold
127
Anti=Ig antibody added to patients blood, RBCs agglutinate if RBCs are covered with Ig
Direct Coombs test
128
normal RBCs added to patient's serum, if serum has anti-RBC surface Ig, they agglutinate
Indirect Coombs
129
Uses for indirect Coombs
Blood typing, screen for maternal Ab in fetal blood
130
interacts with vWF on platelet
GP1b
131
After ADP binds ADP receptor whatmoves to surface
GPIIb/IIIa
132
What binds to GPIIb/IIIa
Fibrinogen
133
after endothelial damage how to blood vessels respond
vasoconstriction (neural stimulation) | -platelets to move slower and stay in area
134
After platelets adhere and bind vWF what do they release
Ca (necessary for coag cascade) and ADP | TXA2 (vasoconstriction and promotes platelet aggregation
135
MOA of aspirin and coag cascade
Apsirin irreverisbily inhibits cyclooxegenase preventing TXA2 production --> no vasoconstriction and harder for platelets to aggregate
136
Clopidogrel, Pasgugrel, Ticagrelor, Ticlopidine
ADP receptor inhibitors - preent expression of glycoproteins IIb/IIIa on sufrace - no release of glycoproteins, staying grey (GRE)
137
Abciximab, Eptifibatide, Tirofiban
Glycoprotein IIb/IIIa inhibitors - bind to the glycoprotein receptor IIa/IIIb on activated platelets, preventing aggregation - FIB (prevent FIBrinogen frombinding)
138
Lifespan of platelet
8-10 days
139
Hemosidinuria and thrombosis
Paroxysmal nocturnal hemoglobinuria
140
Thyroid cells with optically clear nuclei
Papillary thyroid carcinoma
141
Signs of platelet abnormalities
microhemorrhages: mucous membrane bleeding, epistaxis, petechiae, purpura, increased bleeding time, decresed platelet count
142
Defect in GpIb, so platelet can't adhere to vWF
Bernard-Soulier Syndrome
143
Defect in GpIIb/IIIa, defect in platelet to platelet aggreation
Glanzmann Thrombasthenia
144
Thrombocytopenia,Microangiopathic Hemolytic anemia, acute renal failure
Hemolytic-Uremic Syndrome | -Treat with plasmapheresis
145
Anti-GpIIb/IIIa antibodies
Immune thrombocytopenia
146
Incrased megakaryoctytes with microhemorrages
Immune Thrombocytopenia | -Trying to increase platelets because platelet-Ab complex begin consumed by splenic MP
147
Deficiency of ADAMTS13
vWFmetalloprotease --> decreased degradation of vMF multilers --> increased platelet aggregation and thrombosis Thrombotic Thrombocytopenic purpura
148
Fever, anemia, neurological symptoms, thrombocytopenia, renal failure
``` TPP HUNT For The Toilet Paper H: hemolysis U: uremia N: neurological symptoms T: thrombocytopenia TTP ```
149
No platelet aggregationwith ristocetin cofactor assay
von Willebrand Disease - decreased vWF causes increased PTT - defect in platelet plug formation
150
Treatment for von Willebrand disease
Desmopressin
151
Schistocytes, increased d-dimers, decreased fibrinogen, decreaesd factor V and VIII
DIC
152
Causes of DIC
``` STOP Making New Thrombi S: sepsis (G-) T; trauma O: Ob complications P: pancreatitis M: malignancy N: nephrotic syndrome T: transfusion ```
153
Antiplatelet antibodies
Immune thrombocytopenia
154
Most common inherited bleeding disorder
von Willebrand disease

First Aid Yellow (4 decks)

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