Management of acquired von Willebrand syndrome

Acquired von Willebrand syndrome (AVWS) is a rare acquired bleeding disorder that resembles von Willebrand disease by its clinical symptoms and laboratory findings, but differs by its negative personal and family history of bleeding diathesis. AVWS is mostly seen in the elderly, but it has been described in children, often in those with congenital heart disease and Wilms tumor. It is most commonly associated with lymphoproliferative, myeloproliferative, cardio-vascular, or autoimmune diseases, solid tumors, and certain drugs. The diagnosis should be suspected in a patient who is known for one of these underlying conditions and who presents with new onset of bleeding or who will be undergoing an invasive procedure. Treatment of the underlying condition, when possible, usually results in correction of AVWS. When acute bleeding occurs or the underlying condition is not treated, emphasis should be put on control and prevention of bleeding. Many options are available. DDAVP is the first line of treatment for bleeding. vWF concentrates are used to treat bleeding that is unresponsive to DDAVP and as prophylaxis before procedures. This review summarises current knowledge and reviews the different management options for bleeding.