The role of complement system in thrombotic microangiopathy and its research progress
10.3760/cma.j.issn.1009-9158.2019.12.005
- VernacularTitle: 补体系统在血栓性微血管病中的作用及研究进展
- Author:
Hui CAO
1
;
Jun WU
;
Yujing ZHAO
Author Information
1. Department of Clinical Laboratory, Beijing Jishuitan Hospital, Beijing 100035, China
- Publication Type:Journal Article
- Keywords:
Thrombotic microangiopathies;
Complement pathway, alternative;
Complement activation;
Complement system proteins;
Biomarkers
- From:
Chinese Journal of Laboratory Medicine
2019;42(12):998-1001
- CountryChina
- Language:Chinese
-
Abstract:
Thrombotic microangiopathy (TMA) is a group of acute clinical pathological syndromes with common pathological features, which include hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura syndrome. They have many similarities in etiology and clinical presentation. The role of abnormal activation of complement bypass pathway in the genesis and development of HUS has been recognized. More than 100 kinds of complement regulatory factors or gene mutations of complement itself were found to be associated with the development of HUS, which resulted in the decrease of negative complement regulatory protein activity or the increase of complement activation protein function. Abnormal activation of complement system resulted in endothelial injury and thrombosis. Loss of ADAMTS13 activity (<10%) is the most important pathogenesis of TTP. However, there are more and more evidence that complement bypassing pathway is over-regulated and over-activated in the formation of TTP. At present, the research of TMA is focused on finding specific complement-activated biomarkers in patients with various forms of TMA and developing new targeted therapeutic drugs for the disease.
