Acquired von Willebrand syndrome: focused for hematologists

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, AvWS occurs in persons with no personal and 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 those aspects of greater interest for the hematologist.

Figure 1

Example clinical case of acquired von Willebrand syndrome. Patient’s serum protein electrophoresis. The arrows indicate a double spike within the beta (β) and gamma (γ) regions, respectively.