Saturday, September 15, 2018

Von Willebrand's disease and other bleeding disorders

VWD

Ristocetin induces platelet activity and is used to diagnose von Willebrand's disease, which is most common inherited bleeding/coagulability disorder. Reduced levels of platelet aggregation are seen in vwd in association with ristocetin antibiotic presence.

type 1, 3 -- quantitative defect of VWF
type 2- qualitative defect of VWF

Rx: desmopressin or plasma concentrates c Factor VII and VWF

Bernard Soulier- May Hegglin's anomaly, gray platelet syndrome, inherited giant platelet disorder with thrombocytopenia and bleeding

Hemohilia A-- deficient factor VIII (more common)
Hemophilia B (deficient factor IX ) Christmas disease

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