Research progress of pathogenesis and treatment of hemophagocytic Iymphohistiocytosis / 中华实用儿科临床杂志

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.