Hemophagocytic lymphohistiocytosis, Leishmania and Epstein-Barr infection: an uncommon association

Costa, F; Miranda, A; Figueiredo, A; Correia, P

http://hdl.handle.net/10400.10/2483

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Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of abnormal excessive immune activation, with signs and symptoms of excessive inflammation. Case report: 18-months-old girl admitted for persistent fever for the past two months. No other symptoms associated. History of a trip to the north of Portugal with contact with dogs. On examination: ill-appearance, pale and hepatosplenomegaly. Laboratory findings revealed pancytopenia progressively worst, associated with elevated ferritin (828ng/mL), hypertriglyceridemia (490 mg/dL), elevated soluble interleukin-2-receptor (11045 U/mL), which lead to the diagnosis of HLH. Etiologic investigation: IgM VCA-EBV positive (99U/mL), IgG VCA-EBV negative (<20U/mL), IgG EBNA negative (<3U/mL) and PCR for EBV negative; immunofluorescence and immunoblot serologies for Leishmania spp. were positive. Bone marrow examination did not reveal hemophagocytosis and PCR for Leishmania and EBV was negative. Treatment was initiated with lipossomic amphotericin with progressive improvement of the clinical condition. Discussion: Although serology for Leishmania is not the gold standard for diagnosis, it’s positivity in this case and the epidemiologic history lead to a favourable outcome.