Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Type 1 VWD accounts for 75% of cases and is marked by a quantitive deficiency of von Willebrand factor (VWF), leading to low VWF antigen, VWF activity, and factor VIII levels. Patients with blood type O have VWF levels that are up to 30% lower than those with blood types A, B, or AB.
Most patients (99%) with VWD have mild or asymptomatic disease and never seek clinical attention. However, clinically significant skin and mucosal bleeding can occur. Treatment of bleeding complications is typically achieved with:
Aminocaproic acid is an anti-fibrinolytic agent that is sometimes used for patients with VWD who have menorrhagia or mild mucosal bleeding; it is usually used in combination with other therapies.
Cryoprecipitate is not routinely used for VWD due to the risk of viral transmission; VWF preparations are the preferred therapy for more serious bleeding or prior to a major procedure.
Platelets contain a small proportion of total blood VWF and can be used in patients who have refractory bleeding with VWF concentrates.