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Thromb Haemost 2016; 116(S 01): S18-S25
DOI: 10.1160/TH16-01-0050
5th International Coagulation Meeting 2015 – Barcelona, Spain
Schattauer GmbH

Contemporary issues in the management of von Willebrand disease

Augusto B. Federici
1   Hematology and Transfusion Medicine, L. Sacco University Hospital, Milan, Italy
2   Department of Oncology and Hematology-Oncology, University of Milan, Milan, Italy
,
Christoph Königs
3   Department of Paediatrics, Clinical and Molecular Haemostasis, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany
,
Andra H. James
4   Department of Obstetrics & Gynecology, Duke University, Durham, North Carolina, USA
› Author Affiliations
Further Information

Publication History

Received: 20 January 2016

Accepted after major revision: 22 June 2016

Publication Date:
15 December 2017 (online)

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Summary

Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Bleeding scores in VWD, focused in particular on mucosal bleeding, can be very useful in the diagnosis and validation of different types of treatment. The results of an extended prospective study with a large amount of information on clinical phenotype and implications in treatment are reviewed in this article. Treatment of mucosal and joint bleeding in severe VWD remains difficult in some patients. Due to the lack of data on the use of prophylaxis in these patients it is difficult to establish optimal treatment regimens. An overview of the literature, with a focus on the ongoing PRO.WILL study, is provided here. Furthermore, understanding the changes in von Willebrand factor (VWF) levels during pregnancy is very important for establishing the optimal management strategy for pregnancy and delivery in women with VWD. A recently published prospective observational cohort study in women with and without VWD during the postpartum period provides important data that should allow the improvement of postpartum treatment protocols.

Keywords

von Willebrand disease - prophylaxis - pregnancy - bleeding score - FVIII/VWF concentrates
 

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