Diagnostic Haemostasis, Haematology Department, Institute of Clinical Pathology and Medical Research (ICPMR), Pathology West, Westmead Hospital, Westmead, NSW, Australia.
Haemophilia. 2014 May;20 Suppl 4:94-8. doi: 10.1111/hae.12408.
Inhibitor assays are performed when patients present with unexplained prolonged routine coagulation test times and unexpected and/or unusual bleeding (potential for acquired haemophilia) as well as being a part of normal congenital haemophilia management and monitoring, particularly when bleeding occurs on therapy, or when increments in factor levels post-factor replacement remain lower than expected. In this article, we will describe the assays used, as well as their development, pitfalls in testing such as inter-laboratory variability and false negative/positive results, as well as some strategies for overcoming these pitfalls and potential alternative test approaches. The inter-laboratory coefficient of variation often approaches (and sometimes exceeds) 50%, as evidenced by various external quality assessment groups, and this variability has not improved over recent years. Additional important considerations include appropriate interpretation of test results, repeat testing for confirmation, and assessment of recovery as part of the diagnostic process.
当患者出现不明原因的常规凝血试验时间延长和意外及/或异常出血（潜在获得性血友病）时，以及作为正常先天性血友病管理和监测的一部分时，会进行抑制剂检测，尤其是在治疗期间发生出血时，或因子替代后因子水平的增加低于预期时。在本文中，我们将描述所使用的检测方法，以及它们的发展，检测中的陷阱，如实验室间的变异性和假阴性/阳性结果，以及克服这些陷阱的一些策略和潜在的替代检测方法。正如各种外部质量评估小组所证明的那样，实验室间的变异系数往往接近（有时甚至超过）50%，而且近年来这种变异性并没有改善。其他重要的考虑因素包括对检测结果的适当解释、重复检测以确认以及作为诊断过程的一部分评估恢复情况。
