24th July 2024

Von Willebrand disease (vWD)

This is the most common inherited bleeding disorder. It is inherited via an autosomal pattern with variable penetrance.

There is deficiency or poor function of von Willebrand factor (vWF), which is critical for platelet aggregation at sites of vascular injury. It is also bound to factor VIII, a key component of clotting, which degrades when not bound.

Clinical features of vWD

Presentation is often with mucocutaneous bleeding. Females are more likely to present, commonly with menorrhagia or postpartum bleeding.

Investigations include:

• FBC: anaemia may be present; platelet counts are often normal, although can be reduced
• Coagulation studies: PT is normal; APTT is either prolonged or normal
• vWF antigen level: low

Management of vWD

For minor bleeding, antifibrinolytic treatment such as tranexamic acid can be used, as well as desmopressin, which can stimulate the release of vWF and factor VIII. vWF-containing concentrates are given prior to major procedures or in severe bleeding.