Hemophagocytic lymphohistiocytosis is a rare hematologic disorder caused by dysregulated immune activation and carries a high rate of mortality. It is categorized broadly into Primary (Familial ) and secondary types. The recent classification of histiocytoses by Histiocytic society has placed Hemophagocytic lymphohistiocytosis in the “H” group. Greater awareness of Familial hemophagocytic lymphohistiocytosis is required among clinicians and pathologists for early diagnosis and a better survival. Here we report a case of Familial hemophagocytic lymphohistiocytosis correlating with clinical history, family history, bone marrow findings and genetic tests.
