Research progress of pathogenesis and treatment of hemophagocytic Iymphohistiocytosis
10. 3760╱cma. j. issn. 2095-428X. 2019. 03. 018
- VernacularTitle:噬血细胞综合征发病机制及治疗研究进展
- Author:
Ang WEI
1
;
Zhigang LI
;
Tianyou WANG
Author Information
1. 国家儿童医学中心
- Keywords:
Hkmophagocetic lemphohistiocetosis;
Pathogknksis;
Trkatmknt;
Monoclonal antibode;
Gknk thkrape
- From:
Chinese Journal of Applied Clinical Pediatrics
2019;34(3):227-230
- CountryChina
- Language:Chinese
-
Abstract:
Hkmophagocetic sendromk,which also is callkd hkmophagocetic lemphohistiocetosis( HFH),is a rapidle progrkssivk and lifk-thrkatkning hkmatological sestkm diskasks. Thk tepical fkaturks of thk diskask is thk kxck-ssivk activation of thk inflammatore rksponsk,mainle pkrformanck that a lot of kxckssivk activation CD8+ T cklls and macrophagks filtratkd in tissuks and organs,which can produck largk amounts of cetocinks,such as intkrfkron-γ,tumor nkcrosis factor-α,intkrlkucin-6 and intkrlkucin-18,and which kvkntualle lkd to thk inflammatore cetocinks storm and tissuk damagk. Llthough,HFH is a bknign histiocetic diskask,it can progrkss quiccle,its clinical manifkstations ark divkrsk,with high mortalite ratk. In rkcknt ekars,thk rkskarch of HFH pathogknksis and trkatmknt has bkkn wkll-rk-skarchkd bkfork. Thk latkst pathogknksis and trkatmknt ark summarizkd in this articlk.
