von Willebrand Disease High-yield

von Willebrand Disease High-yield

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@ An autosomal dominant defect.

@ Described by Erik von Willebrand in 1926

@ Source – Weible palade bodiesof endothelial celland alfa granulesof platelets.

@ Deficiency in vWFwith decreased levels of factor VIII

@ Normal plasma vWF level is 10 mg/L, with a half-life of 6-15 hours

@ Themost common inherited bleeding disorder (1% of the population)

@ Most common form of vWD is type 1vWD 

@ Most severe form of vWD is type 3vWD

@ Often presents in childhood with recurrent and prolonged mucosal bleeding (epistaxis, gums, teeth, menorrhagia) 

@ Bleeding occurs after dental(extraction) or surgicalprocedures

@ Often a family historyis present 

@ symptoms worsen with Aspirin use

@ Bleeding time is prolonged

PTandplatelet count will be normal

@ Most accurate test: Ristocetin cofactor assay of patient plasma and vWF antigen level 

Ristocetinis an antibioticthat induces platelet aggregation

Decreased agglutination seen on the ristocetin cofactor assay is diagnostic
of vWD 

@ Bleeding episodes in mild/moderate type 1 disease can be treated with 

DDAVP

@ Menorrhagia can be controlled with OCPs 

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