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Residency Boards > Heme-coag > Flashcards

Heme-coag Flashcards

1
Q

Contents of platelet alpha granules

A

Protein molecules:

Fibrinogen

PDGF

vWF

P-selectin

PF4

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2
Q

Contents of platelet dense bodies

A

Nonprotein molecules ADP ATP serotonin (5-HT) Calcium

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3
Q

GPIb/XI/V complex (CD42)

A

mediates platelt adhesion

Receptor for vWF

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4
Q

GPIIb/IIIa complex (CD41&CD61)

A

Mediates platelet aggregation,

fibrinogen receptor

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5
Q

GPIaIIa

A

Mediates platlet adhesion

Collagen receptors

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6
Q

GPIc/IIa complex

A

Fibronectin receptor

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7
Q

Composition of Heinz body

A

Clumps precipitated hemoglobin

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8
Q

Hermansky-Pudlak Syndrome

A

-abnormal dense granules (normally secrete ADP, ATP, 5-HT and calcium) -Epistaxis, oculocutaneous albinism, pulmonary fibrosis, granulomatous colitis -ceroid-like material within tissue macrophages -common in Puerto rico

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9
Q

hereditary spherocytosis

A

AD inheritance, ANK1 (ankyrin) gene -increased MCHC, and reticulocyte count -PB: spherocytes -sx of extravascular hemolysis Abnormal osmotic fragility and autohemolysis tests (DAT is neg vs AIHA)

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10
Q

hereditary elliptocytosis/ovalocytosis

A

AD, spectrin alpha chain gene mutations -PB: elliptocytes (twice as long as wide) >25% -*hereditary pyropoikilocytosis (HPP): most common in AA, RBCs sensitive to heat *Stomatocytic type (aka southeast asian ovalocytosis): protects against P. vivax

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11
Q

G6PD deficiency

A

*G6PD needed to produce NADPH adn reduced glutathione for protection from oxidants; X-linked recessive inheritance -RBCs hypersensitive to oxidant stress (sulfa drugs, nitrofurantoin, primaquine, fava beans, infection) resulting in episodic hemolysis -p/w extravascular hemolysis -PB: bite cells*, Heniz bodies, poikilocytosis, blister cells

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12
Q

Pyruvate kinase (PK) deficiency

A

-PK catalyzes rate limiting step in glycolysis pathway

AR inheritance

-echinocytes (“burr cells”)

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13
Q

major adult hgb

A

HbA alpha2 beta2

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14
Q

minor adult hgb

A

HBA2 alpha2 delta2

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15
Q

Variant beta chain “S”

A

AA6 Glu–>Val

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16
Q

Variant beta chain “C”

A

AA6 Glu–>Lys

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17
Q

Relative proportion of Hgb types in sickle cell trait

A

SA 35-45% HbS 50-65% HbA <3% HbA2

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18
Q

Relative proportion of Hgb types in sickle cell disease

A

homozygous SS RBC lifespan of 17 days

>80% HbS 1-20% HbF 1-4% HbA2 0% HbA

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19
Q

screening tests for sickle cell

A

metabisulfite dithionate

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20
Q

Right shift O2 binding curve

A

Decreased affinity hb acidosis, increased temp, hyperthermia, increased 2,3 DPG,

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21
Q

Left shift O2 binding curve

A

Increased affinity hb alkalosis, hypothermia, decreased 2,3 DPG

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22
Q

What is Methemoglobin?

A

Hb with iron in ferric (Fe+++) state instead of usual ferrous (Fe++) state -normal low levels of Hi maintained by NADH dependent methemoglobin reductase system -treatment is methylene blue (reduces Hi to Hb)

23
Q

Elevated zinc protoporphyrin

A

Iron deficiency anemia Anemia of chronic disease Lead poisoning

24
Q

Elevated free erythrocyte protoporphyrin

A

Iron def anemia Anemia of chronic dx Lead poisoning

25
Q

Conditions associated with arterial thrombosis

A

Lupus anticoagulant, HIT and hyperhomocysteinemia

26
Q

What molecule is inappropriately high in anemia of chronic disease?

A

Hepcidin, this inhibits iron export from macrophages and enterocytes resulting in functional iron deficiency

27
Q

What supports a diagnosis of anemia of chronic disease versus iron deficiency anemia

A

Elevated serum ferritin

28
Q

Causes of intravascular hemolysis

A

Microangiopathic hemolytic anemia (DIC, HUS, TTP, HELLP) Complement fixation in RBC surface (ABO incompatibility, PNH, PCH) Mechanical heart valves Snake venom Infection (malaria, Vanessa, clostridium)

29
Q

Heinz body composition

A

Insoluble hemoglobin inclusion

30
Q

Pentad of TTP

A

Thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal dysfunction and fever

31
Q

Defect in Glanzmann thrombasthenia

A

decreased or defective GPIIb/IIIa (fibrinogen receptor) resulting in defective platelet aggregation

32
Q

ashkenazi jew with major bleeding after GU surgery

A

Factor XI deficiency

33
Q

The following symptoms are typical of what factor deficiency: bleeding from umbilical stump, poor wound healing, hypertrophic scars.

A

Factor XIII deficiency Dx with urea clot lysis assay

34
Q

What are the green dots? What prep? What disease

A
  • Supravital dye
  • Heinz body (denatured Hgb)
  • G6PD deficiency
35
Q

What 2 coagulation factors are not produced in the liver?

A

VIII-endothelial cells in liver

vWF-endothelial cells and megakaryocytes

36
Q

which type of von willebrand disease results in profound thrombocytopenia and bleeding on exposure to DDAVP?

A

Type IIb

gain of function mutation in GPIb binding domain of vWF

Enhanced ristocetin indudced aggregation

decreased HMW multimers

37
Q

Which type of von willebrand disease is due to loss of function mutations in GPIb binding domain?

A

type IIM

Prevents binding of vWF to GPIb

Multimer analysis normal

38
Q

Which type of von willebrand disease is due to missense mutatino in vWF exon 28?

A

type IIa

Factor 8 and vWF are normal and vWF activity is markedly decreased

39
Q

Which type of von willebrand disease results from a mutation in domain that binds factor 8?

A

Type II N

Mimics hemophila A

40
Q

Which type of von willebrand disease has virtually no vWF?

A

Type III

frameshift, nonsense or deletion mutation in vWF gene

Low factor 8, vWF antigen and activity

41
Q

What two groups of patients (besides hemophiliacs) develop antibodies to factor VIII?

A

elderly

postpartum females

42
Q

What acquired factor deficiency can you find in patients with amyloidosis?

A

Factor 10

43
Q

How does activated protein C inhibit coagulation?

A

Degrades activated factors 5 and 8

44
Q

What factors does antithrombin inhibit?

A

2, 9a, 10a, 11a, 12a

45
Q

What antibody is present in type 2 HIT?

A

anti-PF4 antibody

46
Q

What are two causes of spherocytosis and how do you tell them apart?

A
  1. hereditary spherocytosis: ANK1 (ankyrin) mutations
  2. Autoimmune hemolytic anemia: Positive DAT
47
Q

Does S/alpha increase or decreased hgbS?

A

decrease

48
Q

Does S/beta-thal increase or decrease HbS?

A

Increase (usually >50% HbS)

49
Q

HbD is alpha or beta chain defect?

A

beta

50
Q

HbG is alpha or beta chain defect?

A

alpha

51
Q

Hb Lepore is what sort of hb defect?

A

fusion between alpha and beta genes

52
Q

What two CBC parameters are calculated? What is the formula for each?

A

Hct = MCV x RBC/10

MCHC = Hgb/Hct x 100

53
Q

Will you have large or small platelets in ITP?

A

small

54
Q

What is the half life of factor 13

A

5-10 days (longest of all coag factors)

Residency Boards (9 decks)

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