The impact of bleeding history, von Willebrand factor and PFA-100 (R) on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM-1VWD

Research output: Contribution to journalArticle


P>The relationships between the Platelet Function Analyzer (PFA)-100 and von Willebrand factor (VWF) levels and bleeding score (BS) were evaluated within a multicentre project on Molecular and Clinical Markers for the Diagnosis and Management of type 1 von Willebrand disease (MCMDM-1VWD). PFA-100 closure time, either with epinephrine (EPI) or adenosine diphosphate (ADP)-cartridges, was measured in 107 index cases, 105 affected and 71 unaffected family members, and 79 healthy controls. By regression analysis VWF levels were strongly related to both closure times, with a non-linear progression. In a multiple stepwise regression model, age- and sex-adjusted PFA-100 ADP and VWF ristocetin cofactor activity (VWF:RCo) were independently associated with BS. Most of the variation of BS was predicted by PFA-100 ADP and VWF:RCo alone. In the subgroup of patients with subtle abnormalities of the multimeric pattern, VWF was invariably reduced and closure time prolonged in almost all of them. Neither PFA-100 ADP nor EPI closure times appeared to significantly improve the diagnostic capability of VWF antigen (VWF:Ag) measurement. Thus, in an unselected population a normal PFA-100 would be useful to exclude VWD, but whether it could replace the more specific VWF assay in patients with significant mucocutaneous bleeding symptoms remains to be investigated prospectively.


  • Giancarlo Castaman
  • Alberto Tosetto
  • Anne Goodeve
  • Augusto B. Federici
  • Stefan Lethagen
  • Ulrich Budde
  • Javier Batlle
  • Dominique Meyer
  • Claudine Mazurier
  • Jenny Goudemand
  • Jeroen Eikenboom
  • Reinhard Schneppenheim
  • Jorgen Ingerslev
  • David Habart
  • Frank Hill
  • Ian Peake
  • Francesco Rodeghiero
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Hematology


  • bleeding score, PFA-100 closure time, disorders, inherited bleeding, von Willebrand disease, von Willebrand factor
Original languageEnglish
Pages (from-to)245-251
JournalBritish Journal of Haematology
Issue number3
Publication statusPublished - 2010
Publication categoryResearch

Bibliographic note

The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Emergency medicine/Medicine/Surgery (013240200)