The JAK-STAT pathway and hemophagocytic syndrome
10.3760/cma.j.issn.1673-4408.2019.08.007
- VernacularTitle:JAK-STAT通路与噬血细胞综合征
- Author:
Ang WEI
1
Author Information
1. 国家儿童医学中心
- Keywords:
Hemophagocytic lymphohistiocytosis;
Pathogenesis;
JAK-STAT pathway;
Ruxolitinib;
Treatment
- From:
International Journal of Pediatrics
2019;46(8):566-570
- CountryChina
- Language:Chinese
-
Abstract:
Hemophagocytic syndrome (HPS),also known as hemophagocytic lymphohistiocytosis (HLH),is a syndrome with multiple clinical manifestations and severe impairment of organ function caused by abnormal activation of lymphocytes and histiocytes leading to hypercytokinemia.The pathogenesis of this disease is very complicated.So far,it can not be fully clarified.Studies have shown that activation of JAK-STAT signaling pathway can activate inflammatory factor-related gene transcription,which can cause inflammatory factor cascade reaction,and ultimately lead to the occurrence of hemophagocytic syndrome.In recent years,a series of targeted drugs for JAK-STAT pathway have been developed.A JAK1/JAK2 inhibitor,ruxolitinib,has been found to be useful in the treatment of HLH.This article reviews the mechanism of action of ruxolitinib in HLH and its clinical research results.
