Saccullo, G. and Makris, M. orcid.org/0000-0001-7622-7939 (2016) Prophylaxis in von Willebrand Disease: Coming of Age? Seminars in Thrombosis and Hemostasis, 42 (05). pp. 498-506. ISSN 0094-6176
Abstract
Although in most cases von Willebrand disease (VWD) is a mild disorder, a subgroup of patients experience frequent bleeding. In contrast to severe hemophilia in which prophylaxis is the accepted standard of care, this is less frequently used in VWD. Most type 1 VWD patients can be adequately managed with episodic desmopressin and tranexamic acid. In patients with more severe disease, especially those with type 3 VWD, joint bleeds, epistaxis, menorrhagia, and gastrointestinal bleeding are problematic and usually require treatment with von Willebrand factor/factor VIII (VWF/FVIII) concentrate. While in the past these patients were managed with on-demand VWF/FVIII concentrate, several recent reports have demonstrated the value of prophylactic treatment. Despite some uncertainties about the economic impact of treatment of severe VWD, prophylaxis with VWF concentrate should now be considered as the standard of care for the more severe end of the spectrum of affected individuals. The recent introduction of recombinant VWF concentrate is likely to improve the acceptability of prophylaxis in VWD.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2016 Thieme Publishing. This is an author produced version of a paper subsequently published in Seminars in Thrombosis and Hemostasis. Uploaded in accordance with the publisher's self-archiving policy. |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Faculty of Medicine, Dentistry and Health (Sheffield) > Department of Infection, Immunity and Cardiovascular Disease The University of Sheffield > Sheffield Teaching Hospitals |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 13 Jun 2016 15:25 |
Last Modified: | 30 Jun 2017 20:01 |
Published Version: | http://dx.doi.org/10.1055/s-0036-1581106 |
Status: | Published |
Publisher: | Thieme Publishing |
Refereed: | Yes |
Identification Number: | 10.1055/s-0036-1581106 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:100615 |