Progress of ruxolitinib in the treatment of hemophagocytic lymphohistiocytosis
10.3760/cma.j.issn.1673-4408.2023.04.001
- VernacularTitle:芦可替尼治疗噬血细胞性淋巴组织细胞增生症研究进展
- Author:
Gege ZHANG
1
;
Rui ZHANG
;
Tianyou WANG
Author Information
1. 国家儿童医学中心 首都医科大学附属北京儿童医院血液病中心 儿童血液病与肿瘤分子分型北京市重点实验室 儿科学国家重点学科 儿科重大疾病研究教育部重点实验室 100045
- Keywords:
Hemophagocytic lymphohistiocytosis;
JAK-STAT pathway;
Ruxolitinib
- From:
International Journal of Pediatrics
2023;50(4):219-223
- CountryChina
- Language:Chinese
-
Abstract:
Hemophagocytic lymphohistiocytosis (HLH) is a rare immune-mediated disorder characterized by hyperactivation of antigen-presenting cells and T cells, massive secretion of inflammatory cytokines, and impaired function of natural killer cells and CD8 + T cells.Ruxolitinib is a Januse kinase(JAK)inhibitor that reduces cytokine release and retards the inflammatory response by competitive binding to the JAK catalytic site, to achieve the goal of curing HLH.In recent years, ruxolitinib has been gradually applied in the treatment of HLH, and its effectiveness has also been verified.However, studies have also found that there are efficacy differences in the treatment of HLH caused by different etiologies.This article reviews the mechanism of ruxolitinib in the treatment of HLH and the differences in the efficacy of ruxolitinib in the treatment of HLH of different etiologies.
