Coag 3

Question 1

Defect in Bernard-Soulier

Answer 1

GP Ib/V/IX (CD42)

Question 2

photo of bernard-Soulier

Answer 2

Giant platelet with pseudonucleus

thrombocytopenia

Question 3

labs in bernard-soulier

Answer 3

normal PT/PTT
thrombocytopenia
increased bleeding time
impaired platelet agg with RISTOCETIN

Question 4

defect in Glanzmann

Answer 4

GP IIb/IIIa

Question 5

labs in Glanzmann

Answer 5

normal platelets
impaired platelet agg with ADP, collagen, and Epi
clot retraction test + (no retraction)

Question 6

May-Hegglin mutation

Answer 6

myosin heavy chain 9

Question 7

May-Hegglin photo

Answer 7

Dohle body and giant platelets

little bleeding clinically

Question 8

causes of giant platelets

Answer 8

May-Hegglin
Gray plts
Bernar-Soulier
Montreal
ITP
Mediterranean macrothrombocytosis
Sebastian, Flechner, Epstein, Alport

Question 9

Storage pool deficiency

Answer 9

no 2nd wave with ADP or Epi

Question 10

names of storage pool defects

Answer 10

Gray plt
Quebec 
Wiscott-Aldrich
Cheiad-Higashi
Hermansky-Pudlak
Thrombocytopenia with absent radii

Question 11

small platelets

Answer 11

Wiscott-Aldrich

iron deficiency

Question 12

missing in gray platelet syndrome

Answer 12

alpha granules

Question 13

platelet agg in gray plt

Answer 13

blunted with everything but ADP and epi

Question 14

diseases with no delta granules

Answer 14

Wiskott-Aldrich
Chediak-Higashi
Hermanski-Pudlak

Question 15

“swiss-cheese” platelets

Answer 15

Hermansky Pudlak

Question 16

Plt aff in Aspirin or NSAID

Answer 16

decreased 2nd wave to ADP and epi

no response to collagen and arachdonic acid

Question 17

osler-Weber-rendu syndrome

Answer 17

oral telangiectasias
platelet type bleeding
iron def

Question 18

factor decreased in amyloidosis

Answer 18

factor 10

Question 19

hemophilia A

Answer 19

decreased factor 8

Question 20

hemophilia B

Answer 20

decreased factor 9 (christmas dz)

Question 21

molecular for hemophilia

Answer 21

Xq28

intron 22 inversion

Question 22

bethesda unit to activity

Answer 22

1=50%
2=25%
3=12.5%

Question 23

inhibitors more common in

Answer 23

hemophilia A

Question 24

hemophila C

Answer 24

factor 11 def (mild, AR)

Question 25

tests for factor 13 def

Answer 25

clot stability test

1% monochloracetic acid test

Question 26

deficiency where you can get anaphylaxis from trestment

Answer 26

Factor 9

Question 27

dysfibrinogenemia on mixing study

Answer 27

partial correction

Question 28

platele agg with afibrinogenemia

Answer 28

decreased ADP, epi, AA

Question 29

ddx if plt aff decreased with ADP, Epi, AA

Answer 29

afribinogenemia
Glanzmann
Aspirin
storage pool

Question 30

what vit K does

Answer 30

gamma-carboxylation of glutamine

Question 31

alpha-2-antiplasmin

Answer 31

decreased euglobulin lysis time

normal PT/PTT

Question 32

most common cause of thrombophilia

Answer 32

activated protein C resistance

Question 33

FV leiden mutation

Answer 33

Arg 506 Gln

Question 34

FV cambridge mutation

Answer 34

Arg 306 Threonine

Question 35

how does Protein C deficiency classically present

Answer 35

skin necrosisi with warfarin

neonatal fulminans

Question 36

decreased protein S during stress because

Answer 36

increased C4b (acute phase) with the Protein S binding protein

Question 37

decreased response to heparin

Answer 37

antithrombin III def

Question 38

most common anti-phospholipid specificities

Answer 38

B2-glycoprotein
anticardiolipin
lupus anticoagulant

Question 39

decreased platelets with thrombosis

Answer 39

antiphospholipid syndrome

HIT

Question 40

HIT antibodies to

Answer 40

PF4 in granules

Question 41

homocystinuria

Answer 41

AR, lens dislocaiton, MR, peripheral neuropathy, decreased folate