Von Willebrands Disease

Von Willebrand's disease, which is caused by a deficiency or abnormality of vWF, is the most common inherited bleeding disorder. The vWF is a glycoprotein that is synthesized, stored, and secreted by vascular endothelial cells; it is also found in plasma and platelets. vWF has two functions: (1) in primary hemostasis, vWF allows platelets to adhere to the damaged endothelium; and (2) vWF carries factor VIII in the plasma.

It is estimated that 1 in 100 persons has a gene defective for vWF, which is inherited in an autosomal dominant pattern. However, only 1 in 10,000 manifests a clinically significant bleeding disorder. The most severe type of von Willebrand disease (vWD), known as type III, is very rare, occurring in 1 in 1 million persons; it results from an autosomal recessive defect.

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