perioperative management of von willebrand disease vwd vwf


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.

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