Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantua.
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Haematologica. 2020 Aug;105(8):2032-2037. doi: 10.3324/haematol.2020.255117. Epub 2020 Jun 18.
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.
获得性血管性血友病（AvWS）是一种罕见的出血性疾病，其实验室发现与遗传性血管性血友病疾病相似。然而，与遗传性疾病不同的是，AvWS 发生在没有个人和家族出血史的人群中，并且常与多种潜在疾病相关，最常见的是淋巴增生性、骨髓增生性和心血管疾病。在介绍了一个典型病例后，在这篇叙述性综述中，我们讨论了关于这种获得性出血综合征的病理生理学、临床、实验室和治疗方面的最新数据。我们特别关注对血液学家更感兴趣的那些方面。
