Related information and links

For healthcare personal

von Willebrand disease at a glance

von Willebrand disease (VWD) is a bleeding disorder caused by a quantitative or qualitative defect of von Willebrand factor (VWF), a high molecular weight glycoprotein which plays an essential role in primary haemostasis by promoting platelet adhesion to the subendothelium and platelet aggregation under high shear conditions. Since VWF is also a carrier of factor VIII (FVIII) in plasma, deficiency of VWF results in impairment of both primary haemostasis and of blood coagulation.

In most cases, VWD is congenital, inherited in an autosomal dominant fashion; however, an autosomal recessive pattern is described in some cases. Patients with VWD may have a mild, moderate or severe bleeding tendency that has been present since their early childhood, usually proportional to the degree of the VWF defect.

Patients with a negative family history of bleedings, but with a recent personal history, may have an acquired diathesis similar to congenital VWD, which is often associated with other clinical conditions. In such cases, VWF is synthesised normally, but is cleared too rapidly from the plasma.

Page updated: 2016-06-17