Abstrakt: |
Letterer-Siwe disease is reported in half-brothers, 15 and 11 months old, with a duration of the disease about 2 and 1 month. A similar clinical picture manifested by hyperthermia, hepatosplenomegaly, moderate lymphadenopathy, cutaneous changes in one of the brothers, increasing hypochromic anemia, thrombocytopenia, as well as similar morphologic changes (marked histiocytic infiltration of the viscera, hemorrhages, necroses, neoplastic changes in the liver and lungs of one boy) allowed differentiation of familial hemophagocytic histiocytosis from Letterer-Siwe disease in one family. |