血栓性微血管病的发病机制及诊治进展

    Progress in the pathogenesis, diagnosis and treatment of thrombotic microangiopathy

    • 摘要: 血栓性微血管病(thrombotic microangiopathy,TMA)是一组以微血管性溶血性贫血、血小板减少、缺血性器官损伤为主要表现的临床综合征。TMA主要影响肾脏,但也可能涉及其他系统,如中枢神经系统、心血管系统、呼吸系统和消化系统。TMA的病因复杂多样,可由含1型血小板结合蛋白基序的去整合素样金属蛋白酶成员13活性缺乏或补体失调所致,也可继发于感染、妊娠、药物、自身免疫性疾病、造血干细胞移植等。根据不同的发病机制描述TMA的分类和命名,有助于及时准确地诊断和治疗TMA患者,并最终提高患者的生存率。本文就不同类型TMA的临床表现、发病机制、诊断及治疗做一简要综述。

       

      Abstract: Thrombotic microangiopathy (TMA) is a group of clinical syndromes characterized by microvascular hemolytic anemia (MAHA), thrombocytopenia and ischemic organ damage. TMA mainly affects the kidneys, and also involves other systems, such as the central nervous system, cardiovascular system, respiratory system and digestive system. The etiology of TMA is complicated and diverse, and can be mediated by activity deficiency or complement dysregulation of ADAMTS13 (a metalloproteinase with thromboreactive protein type 1, member 13). TMA can also be secondary to infection, pregnancy, drugs, autoimmune diseases, hematopoietic stem cell transplantation, etc. The classification and naming of TMA based on distinct pathogenic mechanisms contributed to the timely and accurate diagnosis and treatment of TMA, thus improving the survival. This article provided an overview of the clinical presentations, etiology, diagnostic modalities, and therapeutic options for various subtypes of TMA.

       

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