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

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.

Tosetto, Alberto (author)
Goodeve, Anne (author)
Federici, Augusto B. (author)
Lethagen, StefanLund University,Lunds universitet,Institutionen för kliniska vetenskaper, Malmö,Medicinska fakulteten,Department of Clinical Sciences, Malmö,Faculty of Medicine(Swepub:lu)medf-sle (author)
Budde, Ulrich (author)
Batlle, Javier (author)
Meyer, Dominique (author)
Mazurier, Claudine (author)
Goudemand, Jenny (author)
Eikenboom, Jeroen (author)
Schneppenheim, Reinhard (author)
Ingerslev, Jorgen (author)
Habart, David (author)
Hill, Frank (author)
Peake, Ian (author)
Rodeghiero, Francesco (author)
Institutionen för kliniska vetenskaper, MalmöMedicinska fakulteten (creator_code:org_t)

By the author/editor: Castaman, Gianca ...; Tosetto, Alberto; Goodeve, Anne; Federici, August ...; Lethagen, Stefan; Budde, Ulrich; show more...; Batlle, Javier; Meyer, Dominique; Mazurier, Claudi ...; Goudemand, Jenny; Eikenboom, Jeroe ...; Schneppenheim, R ...; Ingerslev, Jorge ...; Habart, David; Hill, Frank; Peake, Ian; Rodeghiero, Fran ...; show less...

About the subject

MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Hematology

Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.