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Hemophagocytic lymphohistiocytosis, Leishmania and Epstein-Barr infection: an uncommon association
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Resumo(s)
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.
Descrição
Palavras-chave
Fever of unknown origin Leishmaniasis Hemophagocytic lymphohistiocytosis
Contexto Educativo
Citação
Revista SPDV. 2018; 14(3): 106-110
Editora
Sociedade Portuguesa de Dermatologia e Venereologia