Advances in the pathogenesis and biomarker of macrophage activation syndrome in children / 国际儿科学杂志

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.