The impact of bleeding history, von Willebrand factor and PFA-100(®) on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM-1VWD
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, 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
Abstract
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.
© 2010 Blackwell Publishing Ltd.
Source: PubMed