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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
Conditions associated with arterial thrombosis
Lupus anticoagulant, HIT and hyperhomocysteinemia
26
What molecule is inappropriately high in anemia of chronic disease?
Hepcidin, this inhibits iron export from macrophages and enterocytes resulting in functional iron deficiency
27
What supports a diagnosis of anemia of chronic disease versus iron deficiency anemia
Elevated serum ferritin
28
Causes of intravascular hemolysis
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
Heinz body composition
Insoluble hemoglobin inclusion
30
Pentad of TTP
Thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal dysfunction and fever
31
Defect in Glanzmann thrombasthenia
decreased or defective GPIIb/IIIa (fibrinogen receptor) resulting in defective platelet aggregation
32
ashkenazi jew with major bleeding after GU surgery
Factor XI deficiency
33
The following symptoms are typical of what factor deficiency: bleeding from umbilical stump, poor wound healing, hypertrophic scars.
Factor XIII deficiency Dx with urea clot lysis assay
34
What are the green dots? What prep? What disease
* Supravital dye * Heinz body (denatured Hgb) * G6PD deficiency
35
What 2 coagulation factors are not produced in the liver?
VIII-endothelial cells in liver vWF-endothelial cells and megakaryocytes
36
which type of von willebrand disease results in profound thrombocytopenia and bleeding on exposure to DDAVP?
Type IIb gain of function mutation in GPIb binding domain of vWF Enhanced ristocetin indudced aggregation decreased HMW multimers
37
Which type of von willebrand disease is due to loss of function mutations in GPIb binding domain?
type IIM Prevents binding of vWF to GPIb Multimer analysis normal
38
Which type of von willebrand disease is due to missense mutatino in vWF exon 28?
type IIa Factor 8 and vWF are normal and vWF activity is markedly decreased
39
Which type of von willebrand disease results from a mutation in domain that binds factor 8?
Type II N Mimics hemophila A
40
Which type of von willebrand disease has virtually no vWF?
Type III frameshift, nonsense or deletion mutation in vWF gene Low factor 8, vWF antigen and activity
41
What two groups of patients (besides hemophiliacs) develop antibodies to factor VIII?
elderly postpartum females
42
What acquired factor deficiency can you find in patients with amyloidosis?
Factor 10
43
How does activated protein C inhibit coagulation?
Degrades activated factors 5 and 8
44
What factors does antithrombin inhibit?
2, 9a, 10a, 11a, 12a
45
What antibody is present in type 2 HIT?
anti-PF4 antibody
46
What are two causes of spherocytosis and how do you tell them apart?
1. hereditary spherocytosis: ANK1 (ankyrin) mutations 2. Autoimmune hemolytic anemia: Positive DAT
47
Does S/alpha increase or decreased hgbS?
decrease
48
Does S/beta-thal increase or decrease HbS?
Increase (usually \>50% HbS)
49
HbD is alpha or beta chain defect?
beta
50
HbG is alpha or beta chain defect?
alpha
51
Hb Lepore is what sort of hb defect?
fusion between alpha and beta genes
52
What two CBC parameters are calculated? What is the formula for each?
Hct = MCV x RBC/10 MCHC = Hgb/Hct x 100
53
Will you have large or small platelets in ITP?
small
54
What is the half life of factor 13
5-10 days (longest of all coag factors)

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