Von Willebrand Disease and Hemorrhagic Abnormalities of Platelet and Vascular Function

Von Willebrand disease, which is the most common inherited bleeding disorder, affects about 0.1% the population. A careful bleeding history can guide specialized testing to determine the specific type of von Willebrand disease. Treatments include desmopressin, which stimulates release of the patient’s own von Willebrand factor, and infusion of von Willebrand factor concentrates. Tranexamic acid is useful for acute bleeding episodes. Acquired von Willebrand disease syndrome is often associated with paraproteinemias and hematologic malignancies, and its treatment focuses on ameliorating the precipitating condition. Functional platelet defects can be congenital or acquired, usually related to drugs that are used therapeutically as antiplatelet agents. Uncommon primary vascular disorders that lead to bleeding, including hereditary hemorrhagic telangiectasia and Ehlers-Danlos syndrome, currently have no specific effective treatment.