补体系统与血栓性微血管病  

Complement system and thrombotic microangiopathy

作　　者：邹波[1] 杨娉婷[1] ZOU Bo;YANG Ping-ting(Department of Rheumatology,the First Hospital of China Medical University,Shenyang 110001,China)

机构地区：[1]中国医科大学附属第一医院风湿免疫科,辽宁沈阳110001

出　　处：《中国实用内科杂志》2023年第6期441-445,共5页Chinese Journal of Practical Internal Medicine

基　　金：兴辽人才计划项目(XLYC2002062)。

摘　　要：血栓性微血管病(TMA)的基本病理特征为内皮细胞受损、血小板聚集、微血栓形成,进而导致微血管病性溶血性贫血、血小板减少和器官功能障碍。补体具有溶解细胞、调理吞噬作用、参与炎症反应等多种效应。补体过度活化不仅会损伤血管内皮细胞,还会通过与血小板、凝血因子、炎症因子的相互作用促进微血栓形成。因此,补体系统在TMA的发病机制中起着核心作用。文章以非典型溶血尿毒综合征、硬皮病肾危象和HELLP综合征为例,阐述补体系统与TMA之间的紧密关联,旨在帮助临床医生全面地认识TMA发病机制和治疗靶点。Thrombotic microangiopathy(TMA)is a clinical syndrome characterized by endothelial cell damage,platelet aggregation,and microvascular thrombosis,which results in microangiopathic hemolytic anemia,thrombocytopenia,and organ dysfunction.Complement is a part of the immune system that has various functions such as lysing cells,modulating phagocytosis and inflammation.Excessive complement activation not only injures vascular endothelial cells,but also promotes the formation of microvascular thrombi through the interaction with platelets,coagulation factors,and inflammatory mediators.Therefore,the complement system plays an essential role in the pathogenesis of TMA.This article takes atypical hemolytic uremic syndrome,scleroderma renal crisis and HELLP syndrome as examples to illustrate the close relationship between the complement system and TMA,aiming to help clinicians gain a comprehensive understanding of the pathogenesis and treatment targets of TMA.

关 键 词：补体 血栓性微血管病 非典型溶血尿毒综合征 

分 类 号：R543[医药卫生—心血管疾病]