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Case Reports in Hematology
Volume 2015, Article ID 703803, 8 pages
http://dx.doi.org/10.1155/2015/703803
Case Report

Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease

1Department of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UK
2Department of Cardiothoracic Surgery, Imperial College Healthcare NHS Trust, London W2 1NY, UK

Received 19 January 2015; Revised 18 March 2015; Accepted 19 March 2015

Academic Editor: Hugo Guglielmone

Copyright © 2015 Victoria Campbell et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

von Willebrand disease type 3 (VWD3) is a rare but the most severe form of von Willebrand disease; it is due to almost complete lack of von Willebrand factor activity (VWF:RCo). It is inherited as autosomal recessive trait; whilst heterozygote carriers have mild, or no symptoms, patients with VWD3 show severe bleeding symptoms. In the laboratory, this is characterised by undetectable VWF:Ag, VWF:RCo, and reduced levels of factor VIII < 0.02 IU/dL. The bleeding is managed with von Willebrand/FVIII factor concentrate replacement therapy. In this rare but challenging case we report on the successful excision and repair of an ascending aortic aneurysm following adequate VWF/FVIII factor concentrate replacement using Haemate-P.