Von Willebrands Disease is a platelet disorder characterized by a functional defect in Von Willebrands factor (vWF) itself. Because Factor VIII binds to VWF for transport in the blood, people with VWF deficiencies can also have decreased quantities of functional Factor VIII. Although VWF problems affect platelet function, these patients usually have normal platelet counts. As far as genetics and inheritance, both men and women are affected equally.
Von Willebrands factor is essential for platelet binding, therefore, a defect in vWF causes impaired platelet adhesion and aggregation.
The treatment for persons with Von Willebrands Disease who are bleeding involves the administration of the modified antidiuretic hormone, deamino-D-arginine vasopressin (DDAVP). DDAVP can temporarily raise levels of vWF and factor VIII. Cryoprecipitate formerly was the method of treatment but has since been replaced with DDAVP.