Von Willebrand Disease is a platelet disorder characterized by a functional defect in the von Willebrand factor (vWF). 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 average platelet counts. Regarding genetics and inheritance, both men and women are affected equally.
The von Willebrand factor is essential for platelet binding. Therefore, a defect in vWF causes impaired platelet adhesion and aggregation.
Treating bleeding patients with von Willebrand Disease involves administering the modified antidiuretic hormone diamino vasopressin (DDAVP). DDAVP can temporarily raise levels of vWF and factor VIII. Cryoprecipitate was formerly the treatment method but has since been replaced with DDAVP.
