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IFNg is the pivotal mediator in the murine model of primary HLH. Mice given repeated injection with the TLR9 agonist, CpG-containing oligodeoxynucleotides, develop pathology resembling the human disease, sHLH. Coadministration of an anti-IL-10 Receptor (R) monoclonal antibody (mAb) with CpG induces more severe disease characterized also by hemophagocytosis (fulminant sHLH).

Interferon gamma (IFNg) production is associated to disease parameters in TLR9-induced secondary hemophagocytic lymphohistiocytosis (sHLH) in mice