The occurrence and impact of joint bleeds in Von Willebrand disease

K P Van Galen, Y V Sanders, U Vojinovic, J Eikenboom, M H Cnossen, S Zweegman, R E Schutgens, A G Van Der Bom, K Fijnvandraat, B Laros-Van Gorkom, K Meijer, M R Nijziel, F Leebeek, E P Mauser-Bunschoten

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Background: Von Willebrand disease (VWD) is a heterogeneous inherited bleeding disorder that affects up to 1% of the population. Joint bleeds are not predominant, but have been reported to occur in 8-45% of patients with VWD, especially in those with a more severe phenotype. Joint bleeds can lead to structural joint damage. The most severe type 3 VWD patients develop similar rates of joint range of motion limitation over time as moderate hemophilia A patients. However, the severity, onset and impact of joint bleeds and its complications in VWD patients are largely unknown. Aims: The aim of this study is to assess the incidence, onset and treatment of joint bleeds and its impact on quality of life and joint integrity in moderate and severe VWD. Methods: In the Willebrand in the Netherlands (WIN) study 804 moderate and severe VWD patients (VWF activity 50% in 14/16 VWD patients with joint bleeds who started CFC prophylaxis. We found documented X-ray joint damage in 44% of the patients with joint bleeds compared to 11% of the controls (p
Original languageEnglish
Title of host publicationHaematologica
Number of pages1
Publication statusPublished - 2014

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