The acquired von Willebrand syndrome focused for hematologists

Authors: Franchini, M. and Mannucci, PM.

Publication: Haematologica; June 2020

Affiliations: Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantua; Italy; Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy

Abstract: The acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with laboratory findings similar to those of inherited von Willebrand disease. However, unlike the inherited disease, the AVWS occurs in persons with no personal or family history of bleeding and is often associated with a variety of underlying diseases, most frequently lymphoproliferative, myeloproliferative and cardiovascular disorders. After the presentation of a typical case, in this narrative review we discuss the more recent data on the pathophysiology, clinical, laboratory and therapeutic aspects of this acquired bleeding syndrome. We chose to focus particularly on the aspects of more interest for the hematologist.