Acquired von Willebrand syndrome: focused for hematologists

被引:77
作者
Franchini, Massimo [1 ]
Mannucci, Pier Mannuccio [2 ]
机构
[1] Carlo Poma Hosp, Dept Transfus Med & Hematol, Mantua, Italy
[2] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Angelo Bianchi Bonomi Hemophilia & Thrombosis Ctr, Milan, Italy
关键词
RECOMBINANT FACTOR-VIIA; ESSENTIAL THROMBOCYTHEMIA; RETROSPECTIVE ANALYSIS; VONWILLEBRAND-FACTOR; MULTIPLE-MYELOMA; DISEASE; DESMOPRESSIN; PLASMA; PATHOPHYSIOLOGY; PROTEOLYSIS;
D O I
10.3324/haematol.2020.255117
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:2032 / 2037
页数:6
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