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Semin Thromb Hemost 2018; 44(06): 590-594
DOI: 10.1055/s-0037-1607440
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Alloantibodies in von Willebrand Disease

Massimo Franchini
1   Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantova, Italy
,
Pier Mannuccio Mannucci
2   Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico and University of Milan, Italy
› Author Affiliations
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Publication History

Publication Date:
17 November 2017 (online)

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Abstract

von Willebrand disease (VWD), the most commonly known inherited bleeding disorder, is caused by a partial (type 1) or total (type 3) deficiency or dysfunction (type 2) of von Willebrand factor (VWF). Its management encompasses the prevention or treatment of bleeding by raising endogenous VWF levels using a synthetic agent, such as desmopressin, or providing exogenous VWF concentrates. The development of inhibitory alloantibodies against VWF is a rare but often severe complication encountered during the treatment of type 3 VWD, which is associated with a lack of hemostatic response to infused VWF concentrates and more rarely with allergic, even anaphylactic, reactions. This narrative review will focus on the characteristics of such alloantibodies and their management, which can be very challenging for physicians operating at hemophilia treatment centers.

Keywords

von Willebrand disease - type 3 - alloantibodies - anaphylaxis
 

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