Department of Haematology, UCLH and Haematology Programme, University College London Hospitals Biomedical Research Centre, National Institute for Health Research, London, UK.
Department of Haematology, University Hospital of Wales, Cardiff, UK.
Br J Haematol. 2023 Nov;203(4):546-563. doi: 10.1111/bjh.19026. Epub 2023 Aug 16.
The objective of this guideline is to provide healthcare professionals with clear, up-to-date and practical guidance on the management of thrombotic thrombocytopenic purpura (TTP) and related thrombotic microangiopathies (TMAs), including complement-mediated haemolytic uraemic syndrome (CM HUS); these are defined by thrombocytopenia, microangiopathic haemolytic anaemia (MAHA) and small vessel thrombosis. Within England, all TTP cases should be managed within designated regional centres as per NHSE commissioning for highly specialised services.
本指南的目的是为医疗保健专业人员提供关于血栓性血小板减少性紫癜(TTP)和相关血栓性微血管病(TMA)的管理的清晰、最新和实用的指导,包括补体介导的溶血尿毒综合征(CM HUS);这些疾病的特征是血小板减少、微血管性溶血性贫血(MAHA)和小血管血栓形成。在英格兰,所有 TTP 病例都应按照 NHSE 对高度专业化服务的委托,在指定的区域中心进行管理。