Introduction to Von Willebrand’s Disease

Von Willebrand’s disease is the most common congenital disorder of hemostasis. It is transmitted in an autosomal dominant pattern. It is characterized by deficient or defective von willebrand factor. Von willebrand factor plays a role in platelet adhesion to damaged subendothelium as well as stabilizing factor VIII in plasma.

Clinical Manifestations

• It can affect both males and females.
• Most bleeding is mucosal ( epistaxis, gingival bleeding, menorrhagia and GI bleeding ).
• Bleeding follows minor trauma or surgery.
• Aspirin increases bleeding while pregnancy and estrogen use decreases bleeding.

Investigations

• Bleeding time : Increased
• PT : normal
• APTT : raised
• Factor VIII : low
• vWF : low

In von willebrand’s disease bleeding time is prolonged while in Hemophilia bleeding time is normal.

Treatment

• If mild hemorrhage then give synthetic vassopressin which raises vWF
• If massive hemorrhage then will need factor VIII concentrates that contains considerable quantities of vWF in addition to factor VIII