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18 Cards in this Set

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Defect glycoprotein ,Ib,V ,IX

Bernard soulier disease

Associated with giant plt,abnormal aggregation with ristocetin

Bernard soulier disease

Decrease vWF and glycoprotein Ib

Von willebrand disease

Associated with chromosome 19 ,mark connecting Swizz cheese

Herniasky-pudlak syndrome

Decrease plt dense granule

Herniasky-pudlak syndrome

Chromosome 13

Chediak higashi

Associated with primary and secondary granules giant lysosomes

CHS

CHS is present in

Albinism ,abnormal pigment, repeated plt

Decrease in number of granules ,small plt

WAS

Triad for recurrent infection,eczema,dec plt

Was

Aggregation pattern response

Dec aggregation response ADP,collagen,epinephrine


Normal response to thrombin

Dec atp-adp ratio

Storage pool disease

Deficiency of alpha granules ,large plt

Gray plt syndrome

Lab finding of gray plt syndrome

Prolong BT


Thrombocytopenia


ThrombocytopeniaFibrosis of bmLarge plt


Fibrosis of bm


Large plt

Thrombocytopenia

<100,000/mm3

Due to excessive deposition of platelet aggregation in renal and cerebral vessel

Thrombotic thrombocytopenic purpura

Believed due to vascular wall dysfunction

TTP

TTP is common in

Woman