Von Willebrand Disease

Von Willebrand disease is the most common hereditary bleeding disorder. The disease is caused by inherited defects in the concentration, structure, or function of von Willebrand protein, a multimeric protein that mediates the initial adhesion of platelets at sites of vascular injury and binds and stabilizes blood clotting factor VIII in plasma. Defects in von Willebrand factor concentration, structure, or function that were not inherited can also occur; this is termed acquired von Willebrand syndrome. These defects occur as a consequence of other medical disorders (valvular heart disease, thrombocythemia, malignant neoplasms, and myeloproliferative and autoimmune diseases). This review examines the laboratory evaluation, clinical variants, and treatment of von Willebrand disease. Figures show the initial assessment of von Willebrand disease, and a treatment algorithm for von Willebrand disease. Tables list types of von Willebrand disease and medications used to treat von Willebrand disease. This review contains 2 highly rendered figures, 2 tables, and 10 references.