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

Eva M. de Wee; Yvonne V. Sanders; Eveline P. Mauser-Bunschoten; Johanna G. van der Bom; Manon E. L. Degenaar-Dujardin; Jeroen Eikenboom; Arja de Goede-Bolder; Britta A. P. Laros-van Gorkom; Karina Meijer; Karly Hamulyák; Marten R. Nijziel; Karin Fijnvandraat; Frank W. G. Leebeek; for the WiN study group

doi:10.1160/TH12-04-0244

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2012; 108(04): 683-692
DOI: 10.1160/TH12-04-0244

Blood Coagulation, Fibrinolysis and Cellular Haemostasis

Schattauer GmbH

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

Eva M. de Wee

¹Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands

,

Yvonne V. Sanders

¹Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands

,

Eveline P. Mauser-Bunschoten

²Van Creveldkliniek/Department of Hematology, University Medical Center Utrecht, Utrecht, The Netherlands

,

Johanna G. van der Bom

³Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands

⁴Jon J van Rood Center for Clinical Transfusion Medicine, Sanquin Research, Leiden, The Netherlands

,

Manon E. L. Degenaar-Dujardin

⁵Netherlands Hemophilia Society, Badhoevedorp, The Netherlands

,

Jeroen Eikenboom

⁶Department of Thrombosis and Haemostasis, Leiden University Medical Center, Leiden, The Netherlands

⁷Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, Leiden, The Netherlands

,

Arja de Goede-Bolder

⁸Department of Pediatric Hematology and Oncology, Erasmus University Medical Center/Sophia Children’s Hospital, Rotterdam, The Netherlands

,

Britta A. P. Laros-van Gorkom

⁹Department of Hematology, Radboud University Nijmegen Medical Centre, The Netherlands

,

Karina Meijer

¹⁰Department of Hematology, University Medical Center Groningen, Groningen, The Netherlands

,

Karly Hamulyák

¹¹Department of Hematology, University Hospital Maastricht, Maastricht, The Netherlands

,

Marten R. Nijziel

¹²Department of Internal Medicine, Maxima Medisch Centrum, Eindhoven, The Netherlands

,

Karin Fijnvandraat

¹³Department of Pediatric Hematology, Emma Children’s hospital, Academic Medical Center, Amsterdam, The Netherlands

,

Frank W. G. Leebeek

¹Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands

,

for the WiN study group

› Author Affiliations

Abstract

Summary

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.

Keywords

Bleeding - bleeding phenotype - von Willebrand disease - von Willebrand factor

Full Text

References

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