Advance in pathogenesis, diagnosis and treatment of HLH associated with rheumatic disease in children / 国际儿科学杂志

ABSTRACT

Hemophagocytic lymphohistiocytosis(HLH)is a critical hematological disorder characterized by immune dysfunction with multiple organs and systems involved.HLH secondary to rheumatic disease is also named as macrophage activation syndrome(MAS). In children, MAS is often covered by primary diseases so that it is hard to be discovered early, associated with critical condition and the difficulty of diagnosis and treatment.In this review, we summarize the advances on pathogenesis, diagnosis and treatment of MAS to improve the understanding of MAS and contribute to clinical diagnosis and treatment.