von Willebrand Disease

This disorder results from a deficiency or defect in the protein known as von Willebrand Factor (vWF). It is named after the Swedish clinician who first discovered the disease. vWF is involved in more than one stage of blood clotting. It is the protein that binds platelets to damaged tissue and promotes platelet aggregation at the earliest stage of clot formation. It also acts as a stabiliser of Factor VIII. In plasma, Factor VIII is bound to vWF which protects it and stops its breakdown.

vWD is quite a common condition, and as many as 1 in 100 of the population may be mildly affected, although most are not aware of it because they have no serious symptoms. Fortunately, a large majority of those affected do not have the most severe form of vWD. The disorder is not sex-linked and both males and females may be affected. The bleeding problems experienced by vWD patients are often mucosal (nose bleeds, gastrointestinal bleeds, heavy menstrual bleeding for women, etc.). Some people with vWD receive treatment with the hormone analogue DDAVP (which is not a blood product). Where DDAVP is not effective, then replacement using plasma-derived vWF concentrates takes place.

Factor VIII/vWF Concentrate - Patient Information Leaflet (PIL)