Acquired Von Willebrand Syndrome: A Review

Abstract

Acquired von Willebrand Disease (AvWD) is an uncommon bleeding disorder resulting from the development of decreased quantities of circulating functional von Willebrand factor (vWF). The condition may occur due to a number of pathogenic mechanisms, and is invariably associated with an underlying disorder such as congenital heart disease, neoplasms, and autoimmunity or with the use of certain drugs. While its manifestations are similar to that of inherited von Willebrand disease (vWD) in terms of clinical signs and laboratory results, it differs in that the patient lacks a personal or family history of bleeding and presents later in life. Management of the disorder is based on treatment of the underlying condition, which usually results in resolution of the bleeding diathesis. Acute hemorrhage usually requires vWF concentrates and in some cases immunosuppression. Other options involve use of Intravenous Immunoglobulins (IVIg) or plasmapheresis. Recently, immunotherapies and novel agents such as Emicizumab, Lenalidomide and Rtiuximab have been tried as off-label options.