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 (1) , Alberto Tosetto (1) , Anne Goodeve (2) , Augusto B Federici (3) , Stefan Lethagen (4) , Ulrich Budde (5) , Javier Batlle (6) , Dominique Meyer (7) , Claudine Mazurier (8) , Jenny Goudemand (9) , Jeroen C.J. Eikenboom (10) , Reinhard Schneppenheim (11) , Jorgen Ingerslev (12) , David Habart (13) , Frank Hill (14) , Ian R Peake (15) , Francesco Rodeghiero (1)

1 Department of Cell Therapy and Hematology
2 Haemostasis Research Group, Department of Cardiovascular Science
3 Hemophilia and Thrombosis Centre
4 Department for Coagulation Disorders
5 Hemostaseology, Medilys Laborgesellschaft mbH
6 Servicio de Hematologia y Hemoterapia
7 Hémostase et thrombose
8 du Fractionnement et des Biotechnologies
9 Laboratoire d'Hematologie
10 Hematology, C2-R
11 Department of Pediatric Hematology and Oncology
12 Centre for Hemophilia and Thrombosis
13 and Blood Transfusion
14 Department of Hematology
15 School of Medicine

We evaluated the relationships of Platelet Function Analyzer (PFA)-100 with von Willebrand factor (VWF) levels and bleeding score (BS) within a multicenter 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 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 with 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 is 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.

Mots clés: Medicine

Giancarlo Castaman, Alberto Tosetto, Anne Goodeve, Augusto B Federici, Stefan Lethagen, et al.. 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, 2010, 151 (3), pp.245. ⟨10.1111/j.1365-2141.2010.08333.x⟩. ⟨hal-00569410⟩