The paper reports the case of a previously healthy 4-year-old-girl who presented with pallor, fever and hepatosplenomegaly. Laboratory findings included pancytopenia, hypertriglyceridemia and hyperferritinemia. Initial diagnosis of kala-azar could not be confirmed because of the absence of clinical evidence, negativity of bone marrow aspiration or specific serology for visceral leishmaniasis. Repeated marrow aspiration, performed due lack of clinical response, revealed histiocytes showing haemophagocytosis consistent with haemophagocytic lymphohistocytosis (HLH) and appropriate treatment was started. She continued to have high-grade fever, and a third bone marrow aspiration ultimately revealed presence of Leishmania amastigotes with evidence of active haemophagocytosis. The girl was treated with liposomal amphotericin (AmBisome) for 5 days, following which she recovered rapidly with definitive remission.
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