Coag 3 Flashcards
Defect in Bernard-Soulier
GP Ib/V/IX (CD42)
photo of bernard-Soulier
Giant platelet with pseudonucleus
thrombocytopenia
labs in bernard-soulier
normal PT/PTT
thrombocytopenia
increased bleeding time
impaired platelet agg with RISTOCETIN
defect in Glanzmann
GP IIb/IIIa
labs in Glanzmann
normal platelets
impaired platelet agg with ADP, collagen, and Epi
clot retraction test + (no retraction)
May-Hegglin mutation
myosin heavy chain 9
May-Hegglin photo
Dohle body and giant platelets
little bleeding clinically
causes of giant platelets
May-Hegglin Gray plts Bernar-Soulier Montreal ITP Mediterranean macrothrombocytosis Sebastian, Flechner, Epstein, Alport
Storage pool deficiency
no 2nd wave with ADP or Epi
names of storage pool defects
Gray plt Quebec Wiscott-Aldrich Cheiad-Higashi Hermansky-Pudlak Thrombocytopenia with absent radii
small platelets
Wiscott-Aldrich
iron deficiency
missing in gray platelet syndrome
alpha granules
platelet agg in gray plt
blunted with everything but ADP and epi
diseases with no delta granules
Wiskott-Aldrich
Chediak-Higashi
Hermanski-Pudlak
“swiss-cheese” platelets
Hermansky Pudlak
Plt aff in Aspirin or NSAID
decreased 2nd wave to ADP and epi
no response to collagen and arachdonic acid
osler-Weber-rendu syndrome
oral telangiectasias
platelet type bleeding
iron def
factor decreased in amyloidosis
factor 10
hemophilia A
decreased factor 8
hemophilia B
decreased factor 9 (christmas dz)
molecular for hemophilia
Xq28
intron 22 inversion
bethesda unit to activity
1=50%
2=25%
3=12.5%
inhibitors more common in
hemophilia A
hemophila C
factor 11 def (mild, AR)
tests for factor 13 def
clot stability test
1% monochloracetic acid test
deficiency where you can get anaphylaxis from trestment
Factor 9
dysfibrinogenemia on mixing study
partial correction
platele agg with afibrinogenemia
decreased ADP, epi, AA
ddx if plt aff decreased with ADP, Epi, AA
afribinogenemia
Glanzmann
Aspirin
storage pool
what vit K does
gamma-carboxylation of glutamine
alpha-2-antiplasmin
decreased euglobulin lysis time
normal PT/PTT
most common cause of thrombophilia
activated protein C resistance
FV leiden mutation
Arg 506 Gln
FV cambridge mutation
Arg 306 Threonine
how does Protein C deficiency classically present
skin necrosisi with warfarin
neonatal fulminans
decreased protein S during stress because
increased C4b (acute phase) with the Protein S binding protein
decreased response to heparin
antithrombin III def
most common anti-phospholipid specificities
B2-glycoprotein
anticardiolipin
lupus anticoagulant
decreased platelets with thrombosis
antiphospholipid syndrome
HIT
HIT antibodies to
PF4 in granules
homocystinuria
AR, lens dislocaiton, MR, peripheral neuropathy, decreased folate
