Research Haematology, University College London, London, UK.
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Università degli Studi di Milano, Department of Pathophysiology and Transplantation and Fondazione Luigi Villa, Milan, Italy.
Int J Lab Hematol. 2020 Dec;42(6):685-696. doi: 10.1111/ijlh.13295. Epub 2020 Jul 16.
This guidance document was prepared on behalf of the International Council for Standardization in Haematology (ICSH), by the ADAMTS13 Assay Working Group, which comprises an international group of both clinical and laboratory experts. The document provides recommendations on best practice for the performance of ADAMTS13 assays in clinical laboratories. ADAMTS13 assays support the differential diagnosis of thrombotic microangiopathies and have utility in the management of thrombotic thrombocytopenic purpura (TTP). There are three types of assay: activity, antigen and autoantibody/inhibitor assays. Methods for activity assays differ in terms of sensitivity, specificity, precision and turnaround time. The most widely used assays involve VWF peptide substrates and either chromogenic ELISA or FRET techniques, although chemiluminescence assays and rapid screening tests have recently become available. Tests for autoantibodies and inhibitors allow confirmation of acquired, immune-mediated TTP, while antigen assays may be useful in congenital TTP and as prognostic markers. In this document, we have attempted to describe ADAMTS13 assays and the conditions that affect them, as well as: blood collection, sample processing, quality control, standardization and clinical utility; recognizing that laboratories in different parts of the world have varying levels of sophistication. The recommendations are based on expert opinion, published literature and good clinical laboratory practice.
本指南文件由国际血液学标准化委员会（ICSH）代表 ADAMTS13 检测工作组编写，该工作组由临床和实验室专家组成。本文件就临床实验室进行 ADAMTS13 检测的最佳实践提供了建议。ADAMTS13 检测有助于血栓性微血管病的鉴别诊断，并且对血栓性血小板减少性紫癜（TTP）的管理具有一定价值。目前有三种类型的检测方法：活性检测、抗原检测和自身抗体/抑制剂检测。活性检测方法在灵敏度、特异性、精密度和检测周转时间方面存在差异。最广泛使用的检测方法涉及 VWF 肽底物和比色酶联免疫吸附测定（ELISA）或荧光共振能量转移（FRET）技术，尽管化学发光检测和快速筛选检测最近已经问世。自身抗体和抑制剂检测可用于确认获得性免疫介导的 TTP，而抗原检测可能对先天性 TTP 以及作为预后标志物有用。在本文件中，我们尝试描述 ADAMTS13 检测及其影响因素，以及：血液采集、样本处理、质量控制、标准化和临床应用；认识到世界不同地区的实验室具有不同的复杂程度。这些建议是基于专家意见、已发表的文献和良好的临床实验室实践制定的。
