A 5-year-old boy presented with fever and fatigue after a holiday in northern Italy. On physical examination a marked splenomegaly was found. Laboratory investigations showed a pancytopenia as well as several markers suggesting an autoimmune disease. The splenomegaly and pancytopenia continued to progress despite treatment with prednisolone and intravenous immunoglobulins. One and a half years after presentation, the spleen had grown to such an extent that it was causing mechanical problems. Splenectomy was performed for diagnostic and therapeutic purposes. Histological investigation of the spleen showed amastigotes of Leishmania. PCR confirmed the diagnosis visceral leishmaniasis. Leishmaniasis is too often considered to be a tropical disease only. In recent years it has frequently been seen in southern European countries around the Mediterranean Sea.

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