Fire behind the fury: IL-18 and MAS

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T cell-mediated immunity Chapter 8

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Fire behind the fury: IL-18 and MAS by Kenneth L. McClain, and Carl E. Allen Blood Volume 131(13):1393-1394 March 29, 2018 ©2018 by American Society of Hematology

Pathways to hyperinflammation of antigen presenting cell (APC)/macrophage (Mac). Pathways to hyperinflammation of antigen presenting cell (APC)/macrophage (Mac). Models of pathologic inflammation in fHLH (A) and MAS (B). (A) In fHLH, ineffective cytotoxic lymphocyte (T cell [T]) function leads to persistent activation of APC. Failure to prune activated APCs leads to production of IFN-γ, which drives the resulting cytokine storm. (B) The reports1,2 discussed in this review identify mechanisms of MAS in which NLRC4 activation or TLR9 signaling lead to high levels of free IL-18 that stimulate macrophage (Mac) activation and production of IFN-γ, which drives the resulting cytokine storm. Kenneth L. McClain, and Carl E. Allen Blood 2018;131:1393-1394 ©2018 by American Society of Hematology