![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.](https://ash.silverchair-cdn.com/ash/content_public/journal/blood/131/13/10.1182_blood-2018-02-828186/4/blood828186f1.jpeg?Expires=1725169929&Signature=Cgvowxs6g6yLguZwhErDeHbwrQfNyJ-4mO0fhjJ3yZ~CrensMAg~NPOKWACRx5l~MttpF4vsKT76MrL35o4l3dna4zApPCFyF7HYxIemFzyBNzt8~H7DVHg4GE8pJP4qUp6QH8~E7H1COaGazdh7rbIzePD6pnv69cylOHeviUf5I7AqFCpKMISn7aWr6wZM4PChfPZtH40~rLHLiYIEJZQ4J8O9SXqseiXjlLMumLBXu6Rkp9bVo9O1dJtWUaJ2bhjWkj9cDPsfeeX7qRsxgejP4grPy4hnjBbO0FKE~vIGCZo5RNB5YWXW05IRySGtrTvRI8jr8YQip8IzSFZ7zg__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA)
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.
