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补体是免疫系统的重要组成部分,可保护机体防御病原体侵犯。补体活化受精细调控,不适当或不受控制的补体激活可引起局部和(或)全身炎症反应、组织损伤以及疾病。补体参与某些疾病的病理机制很早就被认识到,但其在各类溶血性贫血(hemolytic anemia,HA)疾病中所起作用直至近10年才逐渐清晰,并促进了补体通路抑制剂的研发。本文综述补体在溶血性疾病中的作用,以及补体通路抑制剂用于临床治疗的现状。
Complement is an important part of the immune system that protects the body against pathogens. Complement activation is finely regulated, and inappropriate or uncontrolled complement activation can cause local and / or systemic inflammatory responses, tissue damage, and disease. The pathophysiology of complement involvement in certain diseases has long been recognized, but its role in various types of hemolytic anemia (HA) disease has not been clear until nearly 10 years and has contributed to the development of complement pathway inhibitors R & D. This article reviews the role of complement in hemolytic disease and the status of complement pathway inhibitors for clinical treatment.