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Background Familial hemophagocytic lymphohistiocyosis is a severe inflammatory condition due to genetic defect in cytotoxic activity (e.a. :perforin). This disease is similar to the macrophage activated syndrome (MAS). In this disorder cytotoxic CD8 lymphocytes are highly proliferating due in part to a cytokine storm condition (IFNg mediated). In this inflammatory climate macrophages gets activated and are forming fused cells called histiocytes which are pathognomonic for the disease.

Evaluation of immune suppressive mechanisms in a murine model of familial hemophagocytic lymphohistiocytosis