Favaloro E J, Bonar R, Kershaw G, Sioufi J, Hertzberg M, Street A, Lloyd J, Marsden K
Department of Haematology, Institute of Clinical Pathology and Medical Research, Westmead Hospital, NSW, Australia.
Haemophilia. 2004 May;10(3):232-42. doi: 10.1111/j.1365-2516.2004.00897.x.
Regular multilaboratory surveys of laboratories derived primarily from Australia, New Zealand and Southeast Asia have been conducted over the past 7 years to evaluate testing proficiency in the diagnosis of von Willebrand's disorder (VWD) and to assess changes to test practice. Participating laboratories (currently 45) are asked to perform their usual panel of tests for VWD, and then to self-interpret test results as to the likelihood (or not) of VWD, as well as to the potential subtype identified. Samples provided in the past two survey distributions (both conducted in 2003) were as follows. Survey part A/distribution 1: Normal donor plasma, plasma with borderline normal/reduced levels of VWF (x2) and plasma from an individual with type 2 A VWD. Survey part B/distribution 2 (family VWD study): Plasma from a father, mother and son with borderline normal/reduced von Willebrand factor (VWF), and a daughter with type 3 VWD. In line with previously published survey results, the interassay and within method coefficients of variation (CV) were similar for all assays (around 15-25%), although tending to be slightly higher for VWF:RCo and VWF:CB than VWF:Ag and FVIII:C. Most laboratories reported test values consistent with expected findings, and made correct interpretations or predictions regarding the nature of the samples, although discrepant assay results or interpretations are still seen in approximately 5-10% of responses (typically from laboratories using a more limited test panel or not performing the VWF:CB). Overall, problems with the non-identification of functional VWF discordance in type 2 VWD, the misidentification of functional VWF discordance in type 1 VWD, and difficulties in discriminating types 1 and 3 VWD appear to predominate. In comparison with previous surveys, performance of electro-immuno diffusion (EID) (or Laurel gel) procedures has now ceased, and a reduction in VWF:RCo and VWF:Multimer testing and an increase in latex immunoassay (LIA) testing is sustained. We conclude that laboratories are generally proficient in tests for VWD, and that diagnostic error rates are reduced when test panels are more comprehensive and include the VWF:CB.
在过去7年中,主要对来自澳大利亚、新西兰和东南亚的实验室进行了定期的多实验室调查,以评估血管性血友病(VWD)诊断中的检测熟练度,并评估检测方法的变化。要求参与的实验室(目前有45个)对VWD进行常规检测,然后自行解读检测结果,判断VWD的可能性以及所识别的潜在亚型。过去两次调查(均在2003年进行)提供的样本如下。调查A部分/第1次分发:正常供体血浆、血管性血友病因子(VWF)水平临界正常/降低的血浆(2份)以及2A型VWD个体的血浆。调查B部分/第2次分发(家族性VWD研究):VWF水平临界正常/降低的父亲、母亲和儿子的血浆,以及3型VWD女儿的血浆。与先前公布的调查结果一致,所有检测的批间和方法内变异系数(CV)相似(约15 - 25%),尽管VWF:RCo和VWF:CB的变异系数往往略高于VWF:Ag和FVIII:C。大多数实验室报告的检测值与预期结果一致,并对样本性质做出了正确的解读或预测,不过在大约5 - 10%的回复中仍可见到不一致的检测结果或解读(通常来自检测项目较有限或未进行VWF:CB检测的实验室)。总体而言,2型VWD中功能性VWF不一致未被识别、1型VWD中功能性VWF不一致被错误识别以及区分1型和3型VWD存在困难等问题似乎较为突出。与之前的调查相比,目前电免疫扩散(EID)(或Laurel凝胶)方法已不再使用,VWF:RCo和VWF:多聚体检测减少,乳胶免疫测定(LIA)检测增加。我们得出结论,实验室在VWD检测方面总体上较为熟练,当检测项目更全面并包括VWF:CB时,诊断错误率会降低。