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HLH Pathogenesis of HLH HLH is a rare and potentially life-threatening hyperinflammatory response syndrome characterized by dysregulated activation of cytotoxic T-lymphocytes, NK-cells, and macrophages resulting in a cascade of hypercytokinemia and immune-mediated harm to various organ systems (Fig

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Then, they have validated key findings in vitro and in vivo in Cftr -KO mice and ex vivo in CF patient-derived endothelial cells