The guideline group was selected to be representative of UKbased medical experts. MEDLINE and EMBASE were searched systematically for publications in English, using the keywords: thrombotic thrombocytopenia purpura (TTP), ADAMTS13, plasma exchange (PEX) and relevant key words related to the subsections of this guideline. The writing group produced the draft guideline, which was subsequently revised by consensus by members of the Haemostasis and Thrombosis Task Force of the BCSH. The guideline was then reviewed by a sounding board of British haematologists, the BCSH and the British Society for Haematology Committee and comments incorporated where appropriate. The ‘GRADE’ system was used to quote levels and grades of evidence, details of which can be found at http://www.bcshguidelines.com.

The objective of this guideline is to provide healthcare professionals with clear, up-to-date, and practical guidance on the management of TTP and related thrombotic microangiopathies, defined by thrombocytopenia, microangiopathic haemolytic anaemia (MAHA) and small vessel thrombosis.

选择指南小组代表英国医学专家。系统检索 MEDLINE 和 EMBASE 中的英文出版物，使用关键词：血栓性血小板减少性紫癜 (TTP)、ADAMTS13、血浆置换 (PEX) 和与本指南小节相关的相关关键词。编写小组编写了指南草案，随后由 BCSH 止血和血栓形成工作组成员以协商一致方式进行了修订。然后由英国血液学家、BCSH和英国血液学会委员会的声音委员会审查该指南，并在适当时纳入评论。“GRADE”系统用于引用证据等级和等级，其详细信息可参见http://www.bcshguidelines.com

本指南的目的是为医疗保健专业人员提供 TTP 和相关血栓性微血管病（定义为血小板减少症、微血管病性溶血性贫血 (MAHA) 和小血管血栓形成）管理的清晰、最新和实用指南。