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Undetected factor VIII in a patient with type 3 von Willebrands disease mistaken as severe haemophilia A

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Abstract:

Summary. 

von Willebrand disease (VWD) type 3 is a rare disorder characterized by absent or <0.1 UmL−1 of ristocetin cofactor (VWF:RCo), and a very low level of factor VIII (FVIII:C). A total absence of FVIII:C has never been reported in type 3 VWD. This case illustrates the effect of severe von Willebrand factor (VWF) deficiency on the factor VIII level.

Keywords: factor VIII level; severe haemophilia A; type 3 von Willebrand disease

Document Type: Research Article

DOI: http://dx.doi.org/10.1111/j.1365-2516.2009.02062.x

Affiliations: 1: Department of Pediatric Hematology-Oncology, McMaster University, Hamilton, ON, Canada 2: Department of Pathology and Molecular Medicine, Queen’s University, Kingston, ON, Canada 3: Hamilton Health Sciences, Hamilton, ON, Canada 4: Department of Medicine, McMaster University

Publication date: November 1, 2009

bsc/haem/2009/00000015/00000006/art00011
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