Determinants of bleeding phenotype in adult patients with moderate or severe von Willebrand disease

EM Wee, Yvonne Sanders, EP Mauser-Bunschoten, JG (Anske) van der Bom, MEL Degenaar-Dujardin, J Eikenboom, A Bolder, BAP Laros-Van Gorkom, K (Karina) Meijer, K Hamulyak, MR Nijziel, K Fijnvandraat, Frank Leebeek

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We performed a nation-wide cross-sectional study to evaluate determinants of bleeding symptoms in a large unselected cohort of adults with von Willebrand disease (VWD). VWD patients were included (n=664), based on lowest historically measured VWF:Ag and VWF:Act levels <= 30 U/dl. Menorrhagia (85%), cutaneous bleeding (77%), bleeding from minor wounds (77%) and oral-cavity bleeding (62%) occurred most frequently. Higher age was associated with a higher bleeding score (BS), determined according to Tosetto, in females. A 10 year increase in age was associated with 0.8 point (95% confidence interval [CI] 0.4-1.1) higher BS. Females had higher BS than males (median 12 vs. 10, p=0.012). BS differed significantly between VWD type 1, 2 and 3: median 9 (-2-31), 13 (-1-33) and 19.5 (1-35), respectively (p < 0.001). BS was strongly associated with VWF and FVIII levels: individuals with VWF:Ag levels <= 10 IU/dl, VWF:Act <= 10 IU/dl and FVIII:C <= 10 IU/dl had, respectively, 5.3 point (95%CI 3.2-7.3), 4.3 point (95%CI 2.9-5.8) and 9.6 point (95%CI 6.5-12.7) higher BS, than those with levels > 30 IU/dl. In type 3 patients 1 IU/dl FVIII:C decrease was associated with 0.6 point (95%CI 0.1-1.1) BS increase (p=0.021). In conclusion, in VWD patients the bleeding phenotype is strongly associated with type of VWD and VWF and FVIII levels.
Original languageUndefined/Unknown
Pages (from-to)683-692
Number of pages10
JournalThrombosis and Haemostasis
Issue number4
Publication statusPublished - 2012

Research programs

  • EMC COEUR-09
  • EMC MM-04-54-08-A

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