Von Willebrand's disease (vWD) is a hereditary clotting disorder that arises from a deficiency of von Willebrand factor (vWF), known to affect humans and dogs. It is a disease caused by problems with platelet adhesion.
In humans, the incidence of vWD is roughly about 1 in 1000 individuals.
There are three described types of vWD, type I, type II and type III, of which type I and II are inherited as autosomal dominant traits and type III is inherited as autosomal recessive.
Type I vWD patients have clearly impaired clotting but usually end up leading a nearly normal life (trouble usually arises in the form of dental surgery or troublesome menorrhagia/periods). Type III is the most severe form of vWD and may have severe mucosal bleeding, no detectable vWF antigen, and/or may have sufficiently low factor VIII that they have occasional hemarthroses like mild hemophiliacs.
Patients with vWD normally require no regular treatment. However, they are always at increased risk for bleeding. Prophylactic treatment is sometimes given for patients with vWD who are scheduled for surgery. They can be treated with cyroprecipitate or with Factor VIII. Mild cases of vWD can be trialled on 1-desamino-8-D-arginine vasopressin (DDAVP), which works by raising the patient's own plasma levels of vWf.
Information obtained partly from Harrison's textbook of Internal Medicine, online version, Chapter 177.
List of eponymous diseases
