A case of malignant histiocytosis in a two year old boy is reported. His main clinical features were fever, lymphadenopathy, hepatomegaly and splenomegaly. Lymph node biopsy showed a sinusoidal type of lymph node infiltration, histiocytes of malignant aspect and erythrophagocytosis. Liver infiltration with tumoral cells was demonstrated by needle biopsy. The clinical evolution was rapidly progressive and after six months of chemotherapy he died of intercurrent respiratory infection.
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