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What is Von willebrands disease all are true except -a) Factor VIII C deficiencyb) BT Prolongedc) Normal Ristocetin testd) Defective aggregation and explain in detail?
Von willebrand disease 

Von willebrand disease is characterized by the deficiency of vWF, while the synthesis of factor VIIIc is normal.
vWF has two major function : -


Helps in platelet adhesion
Stabilizes factor VIIIc.


So, deficiency of vWF results in manifestation of both.

1. Platelet adhesion defect

Bleeding from small venules and capillaries of skin and mucous membrane e.g., epistaxis.
Menorrhagia and GI bleeding.
Prologed bleeding time

2. Coagulation defect (due to factor VIII dysfunction)

Bleeding into weight bearing joints.
Large post traumatic ecchymoses or hematoma.
Prolonged bleeding after any form of surgical procedure or a laceration.
Prolonged PTT.


vWF can be assayed by restocetin agglutination test which measures the ability of restocetin to promote the interaction between vWF and platelet membrane glycoprotein Ib-IX.