Ardillon L, Ternisien C, Fouassier M, Sigaud M, Lefrançois A, Pacault M, Ribeyrol O, Fressinaud E, Boisseau P, Trossaërt M
Laboratory of Hematology and European Haemophilia Comprehensive Care Centre, University Hospital, Nantes, France.
Haemophilia. 2015 Sep;21(5):646-52. doi: 10.1111/hae.12653. Epub 2015 Mar 10.
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.
血小板功能分析仪（PFA - 100）是一种用于探索初级止血功能的生物学工具。该系统已被广泛证明在检测血管性血友病因子（VWF）缺乏方面具有可靠性。然而，大多数研究是基于受益于常规医疗护理和准确诊断的患者，结果可能有些乐观，并未反映其在“现实世界”情况下的表现。我们选择研究PFA - 100筛查VWF瑞斯托霉素辅因子（VWF:RCo）缺乏的可靠性。我们回顾性分析了1997年10月至2013年6月期间转诊至我们中心的4027例患者的结果（n = 6431），这些患者均接受了PFA - Epi、PFA - ADP和VWF:RCo活性评估。我们在一个由213例经基因确诊的血管性血友病患者组成的亚组中研究了血型对结果的影响以及每种方法的性能。我们的经验表明，无论临床情况如何，在“现实世界”条件下，PFA - 100系统都是检测VWF:RCo缺乏的出色筛查试验。阴性预测值（NPV）、阳性预测值、敏感性和特异性分别为：0.98、0.51、0.98和0.40。当使用根据血型调整后的值时，NPV和敏感性低于未根据血型调整的正常值。我们已经表明，PFA - 100方法在筛查VWF缺乏方面比VWF:RCo技术更有效。
