Background and aims Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening disorder characterizedby uncontrolled activation of T-helper 1 lymphocytes and macrophages and overproductionof inflammatory cytokines. Visceral leishmaniasis (VL), is a multisystem infection caused by the dissemination of Leishmania throughout the reticuloendothelial system. HLH associated VL is a rare and difficult to diagnose clinicopathological condition leading to delayed treatment. We report a rare case of HLH associated Visceral Leishmaniasis in a patient who presented with persistent high fever, hepatosplenomegaly and pancytopenia.

Case A 11 month-old baby with persistent high fever for more than 3 weeks and hepatosplenomegaly was assessed. Laboratory testing revealed pancytopenia, hypertriglyceridemia, hyperferritinemia and hypofibrinogenemia. Bone marrow examination revealed hemophagocytosis. A diagnosis of hemophagocytic syndrome was made according to the HLH-2009 diagnostic criteria. Prior to establishing the diagnosis of Leishmaniasis by indirect fluorescent antibody testing, several potential infectious agents were excluded. Treatment with pentavalent antimonials provided relief of all signs and symptoms within ten days.

Conclusion HLH should be included in the differential diagnosis of patients who present with persistent high fever, hepatosplenomegaly and pancytopenia. Besides, physicians should keep in mind Leishmaniasis among the potential etiologic factors of secondary HLH.