von Willebrand disease

von Willebrand disease
von Willebrand disease types I and II are inherited in an autosomal dominant pattern.
Pronunciation
  • /ˌfʌnˈvɪlɪbrɑːnt/
SpecialtyHematology

Von Willebrand disease (VWD) is a type of blood-clotting disorder. It is the most common hereditary coagulopathy in humans, affecting 1% of the population. An acquired form of VWD can sometimes result from other medical conditions. Most people with VWD have no symptoms. Those that do usually have bleeding of varying intensity, including repeated bruising and nosebleeds.

VWD arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a protein required for platelet adhesion through the binding to other proteins, particularly factor VIII. The disease is known to affect several breeds of dogs as well as humans. Four types of hereditary VWD have been described, including platelet-type VWD, with VWD type 1 being the most common. Types 1 and 2 are inherited through an autosomal dominant pattern, meaning at least one parent must also have the disease.

Diagnosis is typically confirmed through blood tests. Managing VWD includes the use of desmopressin after minor trauma or before surgery, which allows the body to release more VWF. The disease is named after the Finnish physician Erik Adolf von Willebrand, who first described the condition in 1926.