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

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.