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。
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.
作者
韩悦
戚嘉乾
HAN Yue;QI Jiaqian(The First Affiliated Hospital of Soochow University,Jiangsu Institute of Hematology,Suzhou,215006,China)
出处
《临床血液学杂志》
CAS
2024年第7期462-467,共6页
Journal of Clinical Hematology
关键词
血栓性血小板减少性紫癜
诊断
治疗
thrombotic thrombocytopenic purpura
diagnosis
treatment
