Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy: Results from the von Willebrand Disease Prophylaxis Network

Elena Holm, Thomas C. Abshire, Joel Bowen, M. Teresa Álvarez, Paula Bolton-Maggs, Manuel Carcao, Augusto B. Federici, Joan Cox Gill, Susan Halimeh, Christine Kempton, Nigel S. Key, Peter Kouides, Alice Lail, Andrea Landorph, Frank Leebeek, Michael Makris, Pier Mannucci, Eveline P. Mauser-Bunschoten, Diane Nugent, Leonard A. ValentinoRochelle Winikoff, Erik Berntorp

Research output: Contribution to journalArticle

Abstract

Clinically, the leading symptom in von Willebrand disease (VWD) is bleeding, chiefly of mucosal type, for example, epistaxis, gingival, or gastrointestinal bleeding, and menorrhagia. In severe forms of VWD with secondary deficiency of factor VIII, spontaneous joint bleeding, resembling that observed in severe haemophilia A, may also be observed. The bleeding patterns of VWD can affect quality of life, and may be life-threatening. The von Willebrand Disease Prophylaxis Network is an international study group formed with the goal of investigating the role of prophylaxis in clinically severe VWD. The objective of the present study is to investigate the response to prophylaxis focusing primarily on epistaxis, joint bleeding, gastrointestinal bleeding, and heavy bleeding associated with menses. Data from 105 subjects, 10 enrolled in a prospective study and 95 in a retrospective study between 2008 and 2013, were available for analysis. The median annualized rate reductions in bleeding were significant for epistaxis (P

Original languageEnglish
Pages (from-to)383-388
Number of pages6
JournalBlood Coagulation and Fibrinolysis
Volume26
Issue number4
DOIs
Publication statusPublished - Jun 6 2015

Keywords

  • bleeding rate
  • epistaxis
  • gastrointestinal bleeding
  • joint bleeding
  • menorrhagia
  • prophylaxis
  • VWD

ASJC Scopus subject areas

  • Hematology
  • Medicine(all)

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    Holm, E., Abshire, T. C., Bowen, J., Álvarez, M. T., Bolton-Maggs, P., Carcao, M., Federici, A. B., Gill, J. C., Halimeh, S., Kempton, C., Key, N. S., Kouides, P., Lail, A., Landorph, A., Leebeek, F., Makris, M., Mannucci, P., Mauser-Bunschoten, E. P., Nugent, D., ... Berntorp, E. (2015). Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy: Results from the von Willebrand Disease Prophylaxis Network. Blood Coagulation and Fibrinolysis, 26(4), 383-388. https://doi.org/10.1097/MBC.0000000000000257