Willebrand disease


Willebrand disease

, von Willebrand disease (vil′ĕ-brant″) [Erik Adolph von Willebrand, Finnish physician, 1870–1949] ,

vWD

A congenital autosomal dominant bleeding disorder caused by a deficiency of von Willebrand factor (a protein that helps platelets stick to injured blood vessels during the formation of blood clots. Inadequate amounts of the protein result in easy bruising or bleeding. Common symptoms of the disorder are frequent bleeding from the gums after brushing the teeth, frequent or prolonged nosebleeds, heavy menstrual periods, or heavy bleeding after childbirth or surgery.

The disease is the most common inherited bleeding disorder. Unlike hemophilia A and B, which are X-linked, it affects both genders. Despite its high prevalence in the population (about 3% of Americans are affected), vWD is often undiagnosed because many affected people have only mild episodes of bleeding. vWD can be diagnosed by the demonstration of low levels of von Willebrand factor in the blood; by a prolonged bleeding time; or by factor VIII deficiency (one function of von Willebrand factor is to carry clotting factor VIII in plasma). Treatments for heavy bleeding episodes (or for necessary surgeries) may include the administration of clotting factors containing von Willebrand factor or factor VIII or synthetic vasopressin.