Advances in the pathogenesis and biomarker of macrophage activation syndrome in children
10.3760/cma.j.issn.1673-4408.2023.02.014
- VernacularTitle:儿童巨噬细胞活化综合征的发病机制与生物标志物研究进展
- Author:
Xiaomeng SHI
1
;
Jun YANG
Author Information
1. 中国医科大学深圳市儿童医院 518038
- Keywords:
Children;
Macrophage activation syndrome;
Biomarker;
Cytokines
- From:
International Journal of Pediatrics
2023;50(2):129-134
- CountryChina
- Language:Chinese
-
Abstract:
Macrophage activation syndrome is a life-threatening syndrome of multiple causes secondary to rheumatic immune diseases.It is characterized by the continuous activation and proliferation of T lymphocytes and macrophages that leads to overwhelming immune response and excessive release of pro-inflammatory mediators, which eventually causes cytokine storm and multiple organ failure.The main clinical manifestations and laboratory abnormalities include fever, hemocytopenia, hepatomegaly, splenomegaly, lymph node enlargement, coagulation disorders, liver function damage, hyperferritinemia, hypertriglyceridemia and the phenomenon of phagocytosis of blood cells in bone marrow.This article reviews the progress of epidemiology, pathogenesis and biomarkers in macrophage activation syndrome to provide new insights for early diagnosis and identification of the complication which has a rapid progress and high fatality rate.
