血栓性血小板减少性紫癜诊治进展

韩悦; 戚嘉乾

doi:10.13201/j.issn.1004-2806.2024.07.005

血栓性血小板减少性紫癜诊治进展

韩悦^1, ,,
戚嘉乾¹

- 苏州大学附属第一医院，江苏省血液研究所(江苏苏州，215006)

详细信息

作者简介:
韩悦，苏州大学附属第一医院血液科副主任、教授、博士生导师。担任中华医学会血液学分会血栓与止血学组副组长，中华医学会血液学分会委员，江苏省血液免疫学会主任委员，中国老年医学会血液学分会常务委员，中华医学会血液学分会白血病学组委员，中国抗癌协会血液分会委员、细胞治疗与移植学组委员，中国整合血液病专委会委员，江苏省“医学重点人才”“六大高峰人才”“333高层次人才”，主持教育部科技进步一等奖、江苏省科技进步一等奖

通讯作者: 韩悦，E-mail：hanyue@suda.edu.cn

中图分类号: R558.2

收稿日期: 2024-03-07

刊出日期: 2024-07-01

Advances in diagnosis and treatment of thrombotic thrombocytopenic purpura

HAN Yue^1, ,,
QI Jiaqian¹

- The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, Suzhou, 215006, China

More Information

Corresponding author: HAN Yue, E-mail: hanyue@suda.edu.cn

Received Date: 07 March 2024

Publish Date: 01 July 2024

摘要

摘要: 血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura，TTP)是一种致命的血栓性疾病，近年来引起了广泛关注。TTP的发病机制与血管性血友病因子裂解酶(ADAMTS13)的缺乏有关。诊断TTP主要依靠临床表现和血浆中ADAMTS13活性的检测。TTP的主要鉴别诊断是溶血性尿毒症综合征和其他类型的血栓性微血管病以及弥散性血管内凝血。目前，血浆置换和利妥昔单抗联合治疗是TTP的主要治疗方案。此外，一些新的药物治疗靶点也在不断涌现，如卡普赛珠单抗、N-乙酰半胱氨酸、硼替佐米和重组ADAMTS13等。展望未来，随着对TTP机制的进一步了解和新药临床试验的进行，相信会有更多有效的诊疗手段出现，目前有创的血浆置换治疗也将会逐步被简便的口服药物替代。
- 血栓性血小板减少性紫癜 /
- 诊断 /
- 治疗
Abstract: Thrombotic thrombocytopenic purpura(TTP) is a life-threatening thrombotic disorder that has attracted widespread attention in recent years. The pathogenesis of TTP is associated with the deficiency of ADAMTS13, a von Willebrand factor-cleaving protease. The diagnosis of TTP primarily relies on clinical manifestations and measurement of ADAMTS13 activity in plasma. The main differential diagnoses of TTP are hemolytic uremic syndrome, other types of thrombotic microangiopathy, and disseminated intravascular coagulation. Currently, plasma exchange and treatment with rituximab are the mainstays of TTP management. In addition, new therapeutic targets have emerged, such as caplacizumab, N-acetylcysteine, bortezomib, and recombinant ADAMTS13. Looking ahead, with further understanding of the mechanisms underlying TTP and ongoing clinical trials of new drugs, it is anticipated that more effective diagnostic and therapeutic approaches will be developed. Non-invasive oral medications may gradually replace invasive plasma exchange as a treatment option.
- thrombotic thrombocytopenic purpura /
- diagnosis /
- treatment

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