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

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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 nonlinear progression. In a multiple stepwise regression model, age- and sexadjusted 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.

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Castaman, G., Tosetto, A., Goodeve, A., Federici, A. B., Lethagen, S., Budde, U., … Rodeghiero, F. (2010). 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. British Journal of Haematology, 151(3), 245–251. https://doi.org/10.1111/j.1365-2141.2010.08333.x

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