Cold agglutinin syndrome and hemophagocytosis in systemic leishmaniasis.

A 3-year-old male child with high fever, hepatosplenomegaly, pancytopenia, haemolysis, striking histiocytosis and hemophagocytosis in bone marrow aspiration and high titre of cold agglutinin is described. Cold agglutinins were defined as polyclonal IgM with anti-I specificity. Diagnosis of visceral leishmaniasis was made on the basis of typical Leishman-Donovan bodies found in the patient's bone marrow, high titre of anti-leishmania antibodies and excellent response to treatment. Visceral leishmaniasis must be considered in the differential diagnosis of diseases with histiocytosis. Cold agglutinin syndrome may contribute to the haemolytic process which exists in leishmania-donovani infection.