Selective IL-2 delivery modulates CD8+ T cells in human HLH and ameliorates disease features in a murine model

Main hemophagocytic lymphohistiocytosis (HLH) is a extreme inflammatory syndrome. In familial HLH, loss-of-function mutations in genes equivalent to Prf1 disrupt perforin-dependent cytolysis, resulting in sustained antigen presentation, uncontrolled T-cell activation, extreme manufacturing of IFN-γ by CD8+ T cells, and collapse of the regulatory T-cell compartment. Antigen-presenting cells should not effectively eradicated. As an alternative, extended immune synapse time leads to persistent stimulation and cytokine launch by CD8+ T cells,1 which in flip drive myeloid activation and systemic irritation.

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