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Review
. 2022 Oct;70(5):566-577.
doi: 10.1007/s12026-022-09285-w. Epub 2022 Jul 11.

Cytokine storm and targeted therapy in hemophagocytic lymphohistiocytosis

Han-Qi Zhang #  1   2 , Si-Wei Yang #  1   2 , Yi-Cheng Fu  1 , Ming-Cong Chen  1 , Cheng-Hao Yang  1   2 , Ming-Hua Yang  1 , Xiao-Dan Liu  3 , Qing-Nan He  4 , Hua Jiang  5 , Ming-Yi Zhao  6
Affiliations
Review

Cytokine storm and targeted therapy in hemophagocytic lymphohistiocytosis

Han-Qi Zhang et al. Immunol Res. 2022 Oct.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening systemic hyperinflammatory syndrome. The central pathogenesis is an explosive cytokine storm characterized by a significant increase in proinflammatory cytokines, including IL-1β, IL-6, IL-18, IFN-γ, and TNF-α. Meanwhile, negative regulatory factors, such as IL-10 and TGF-β, are also related to the production of HLH. Exploring the specific mechanism of cytokine storms could provide ideas regarding targeted therapy, which could be helpful for early treatment to reduce the mortality of HLH. Although some research has focused on the advantages of targeted therapies, there is still a lack of a comprehensive discourse. This article attempts to summarize the mechanisms of action of various cytokines and provide a therapeutic overview of the current targeted therapies for HLH.

Keywords: Cytokine inhibitor; Cytokine storm; Hemophagocytic lymphohistiocytosis; IFN-γ; Targeted therapy.

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