Approaches and options for immunosuppressive treatment of severe hemophagocytic lymphohistiocytosis in children
10.3760/cma.j.issn.1673-4912.2024.11.002
- VernacularTitle:儿童重症噬血性淋巴组织细胞增多症免疫抑制治疗的方法与选择
- Author:
Yiping ZHOU
1
;
Chunxia WANG
;
Yucai ZHANG
Author Information
1. 上海市儿童医院 上海交通大学医学院附属儿童医院重症医学科200062
- Keywords:
Hemophagocytic lymphohistiocytosis;
Macrophage activation syndrome;
Immunotherapy;
Children
- From:
Chinese Pediatric Emergency Medicine
2024;31(11):806-811
- CountryChina
- Language:Chinese
-
Abstract:
Hemophagocytic lymphohistiocytosis（HLH）/macrophage activation syndrome（MAS）are hyperinflammatory diseases that primarily affect children.The main pathophysiological mechanism involves the reduction or lack of T lymphocyte and natural killer cell function，leading to abnormal proliferation and activation of lymphocytes and macrophages，resulting in cytokine storm and organ dysfunction.The main manifestations are high fever，cytopenia，hepatosplenomegaly，elevated ferritin，liver failure，and coagulation dysfunction,with a mortality rate that can exceed 50%.In recent years，HLH/MAS has developed new immunotherapy methods based on the treatment of traditional immunosuppressive agents.This article focused on the clinical research and treatment of new immunotherapy methods.
