Author:
de Wee Eva M.,Sanders Yvonne V.,Mauser-Bunschoten Eveline P.,van der Bom Johanna G.,Degenaar-Dujardin Manon E. L.,Eikenboom Jeroen,de Goede-Bolder Arja,Laros-van Gorkom Britta A. P.,Meijer Karina,Hamulyák Karly,Nijziel Marten R.,Fijnvandraat Karin,Leebeek Frank W. G.,
Abstract
SummaryWe 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.
Funder
Dutch Hemophilia Foundation (Stichting Hemophilia)
CSL Behring (unrestricted grant)
Cited by
85 articles.
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