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Review
. 2019 Nov;187(4):418-430.
doi: 10.1111/bjh.16186. Epub 2019 Sep 9.

How I manage severe von Willebrand disease

Frank W G Leebeek  1 , Ferdows Atiq  1
Affiliations
Review

How I manage severe von Willebrand disease

Frank W G Leebeek et al. Br J Haematol. 2019 Nov.

Abstract

Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Most patients with mild and moderate VWD can be treated effectively with desmopressin. The management of severe VWD patients, mostly affected by type 2 and type 3 disease, can be challenging. In this article we review the current diagnosis and treatment of severe VWD patients. We will also discuss the management of severe VWD patients in specific situations, such as pregnancy, delivery, patients developing alloantibodies against von Willebrand factor and VWD patients with recurrent gastrointestinal bleeding. Moreover, we review emerging treatments that may be applied in future management of patients with severe VWD.

Keywords: severe VWD; treatment; von Willebrand disease.

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Conflict of interest statement

F.W.G. Leebeek received research support from CSL Behring and Shire/Takeda for performing the Willebrand in the Netherlands (WiN) study, and is consultant for uniQure, Novo Nordisk and Shire/Takeda, of which the fees go to the institution. He also received travel support from Sobi and is DSMB member of a study sponsored by Roche. F. Atiq received the CSL‐Behring‐Heimburger Award 2018, and a travel grant from Sobi.

Figures

Figure 1
Figure 1
Management of delivery in severe VWD patients. 1Consists of a haematologist, clinical geneticist, gynaecologist, paediatric haematologist and anaesthesiologist. 2If the causative VWF gene mutation is known in the mother. 3Either physiologically or by factor infusion or desmopressin. 4No invasive management with vacuum or forceps.
See this image and copyright information in PMC

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