Platelet function analyser (PFA‐100) results and von Willebrand factor deficiency: a 16‐year ‘real‐world’ experience

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Platelet function analyser (PFA-100) results and von Willebrand factor deficiency: a 16-year ‘real-world’ experienceL. Ardillon, C. Ternisien, M. Fouassier, M. Sigaud, A. Lefrançois, M. Pacault, O. Ribeyrol, E. Fressinaud, P. Boisseau andM. Trossaërt*Article first published online: 10 MAR 2015

DOI: 10.1111/hae.12653

© 2015 John Wiley & Sons Ltd

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HaemophiliaEarly View (Online Version of Record published before inclusion in an issue)

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How to CiteArdillon, L., Ternisien, C., Fouassier, M., Sigaud, M., Lefrançois, A., Pacault, M., Ribeyrol, O., Fressinaud, E., Boisseau, P. and Trossaërt, M. (2015), Platelet function analyser (PFA-100) results and von Willebrand factor deficiency: a 16-year ‘real-world’ experience. Haemophilia. doi: 10.1111/hae.12653

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Laboratory of Hematology and European Haemophilia Comprehensive Care Centre, University Hospital, Nantes, France

* Correspondence: Dr Marc Trossaërt, Centre Régional de Traitement de l'Hémophilie, 1 Place Alexis RICORDEAU – Centre Hospitalier Universitaire, 44093 Nantes Cedex 1, France.
Tel.: +33 2 40 08 74 68; fax: +33 2 40 08 42 59
e-mail: marc.trossaert@chu-nantes.fr

Publication HistoryArticle first published online: 10 MAR 2015Manuscript Accepted: 19 JAN 2015 SEARCH Search Scope All contentPublication titlesIn this journalIn this issue Search String Advanced >Saved Searches > SEARCH BY CITATION Volume: Issue: Page: ARTICLE TOOLSGet PDF (169K)Get PDF (169K)Save to My ProfileE-mail Link to this ArticleExport Citation for this ArticleGet Citation AlertsRequest Permissions AbstractArticleReferencesCited By View Full Article (HTML) Enhanced Article (HTML) Get PDF (169K)Get PDF (169K) Keywords:platelet function analyser-100;ristocetin cofactor activity;von Willebrand factorSummary

The platelet function analyser (PFA-100) is a biological tool designed to explore primary haemostasis. This system has thus been widely demonstrated as reliable in detecting von Willebrand factor (VWF) deficiency. However, most studies were based on patients benefitting from regular medical care and accurate diagnosis, and it would seem probable that the results were somewhat optimistic, and do not reflect its performances in ‘real-world’ situations. We have chosen to study the reliability of PFA-100 for screening VWF ristocetin cofactor (VWF:RCo) deficiency. We retrospectively analysed the results (n = 6431) of 4027 patients referred to our centre between October 1997 and June 2013 and in whom PFA-Epi, PFA-ADP, and VWF:RCo activity had been evaluated. We studied the influence of blood group on the results and the performances of each method in a subgroup of 213 patients with genetically confirmed von Willebrand disease. We have shown that the PFA-100 system, in our experience, constitutes an excellent screening test for detecting VWF:RCo deficiency, whatever the clinical situation, in ‘real-world’ conditions. The negative predictive value (NPV), the positive predictive value, the sensitivity and the specificity were respectively: 0.98, 0.51, 0.98 and 0.40. When values adjusted for blood group are used, NPV and sensitivity are inferior to those using normal values which have not been adjusted for blood group. We have shown the PFA-100 method to be more efficient in screening for VWF deficiency than the VWF:RCo technique.

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