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

作者：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; Hamulyak Karly; Nijziel Marten R; Fijnvandraat Karin; Leebeek Frank W G^*

来源：Thrombosis and Haemostasis, 2012, 108(4): 683-692.

DOI：10.1160/TH12-04-0244

• Bleeding
• bleeding phenotype
• von Willebrand disease
• von Willebrand factor

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 %26lt;= 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 %26lt; 0.001). BS was strongly associated with VWF and FVIII levels: individuals with VWF:Ag levels %26lt;= 10 IU/dl, VWF:Act %26lt;= 10 IU/dl and FVIII:C %26lt;= 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 %26gt; 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.

• 出版日期2012-10