Von Willebrand's disease

Definition:

A hereditary bleeding disorder caused by moderate-to-severe factor VIII deficiency and low-levels of factor VIII-related antigen (substances necessary for blood clotting). Additionally, there is insufficient von Willebrand factor which also helps blood clot. The Von Willebrand factor helps platelets to stick to the blood vessel wall and to each other, which is necessary for normal blood clotting.

Causes, incidence, and risk factors:

Von Willebrand's disease is the most common hereditary bleeding disorder. It is transmitted by an autosomal gene and affects both sexes. Most cases are mild and bleeding may occur after a surgical procedure or tooth extraction. The condition is worsened by the use of aspirin and other nonsteroidal anti-inflammatory drugs. Bleeding may decrease during pregnancy.

The disease is estimated to occur in 3 or 4 people per 100,000. There are no racial or ethnic associations with this disorder. A family history of a bleeding disorder is the primary risk factor.