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VON WILLEBRAND DISEASE

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VON WILLEBRAND DISEASE

Most common hereditary bleeding disorder

Von Willebrand Disease (VWD) is the most common hereditary bleeding disorder in, with an estimated prevalence ranging upward to 1% of the general population. Males and females are both approximately equally affected. VWD arises from a deficiency or dysfunction of von Willebrand factor (VWF).

VWD is a partial quantitative deficiency of normally functioning VWF. It is the most common variant, accounting for approximately 75% of symptomatic individuals, and is generally associated with mild bleeding symptoms. Type 2 VWD is a qualitative deficiency due to abnormal function of VWF. It accounts for nearly all the remaining affected individuals

It has further subtypes including 2A, 2B, 2M, and 2N. These subtypes are characterized by where the abnormal function occurs within the VWF. Of the subtypes, 2A is the most common.

TYPE 3

VWD is the rarest of the variants and is estimated to only affect 1 in 1,000,000 individuals. It arises from the virtual absence of VWF protein and can be associated with severe bleeding symptoms.

We use medications to increase plasma level of VWF and factor 8. We sometimes use plasma transfusion and factors to increase levels. Medications are used to decrease the risk of bleeding. Specific of management of VWD with AVWS, menorrhagia, hemorrhagic ovarian cysts, pregnancy and childbirth IS ALSO NEEDED UNDER EXPERT SUPERVISION.