A boy, who started remittent fever reaching 40°C at the age of 4 years and 9 months and gradually developed hepatosplenomegaly, lymphadenopathy, pancytopenia, and hypergammaglobulinemia, is reported. Aspirated bone marrows contained sea‐blue histiocytes, and nitroblue tetrazolium test was abnormal. X‐ray examination revealed a tumor mass in the right lung, uremia occurred terminally, and he died at the age of 10 years and 3 months. Surgical specimens, obtained by splenectomy, and necropsy revealed the presence of characteristic sea‐blue cells in the bone marrow, spleen, lymph nodes, liver, and kidneys, and pseudotuberculous granulomas with or without aspergillus in the lungs, epicardium, liver, and lymph nodes. Histochemically, sea‐blue granules accumulating in the cytoplasm of the histiocytes were proved to be a lipogenic ceroid‐like pigment. Electron microscopically, these granules were comprised of round or ovoid, membrane‐bound or free lipid droplets of various sizes and electron densities, occasionally containing poorly oriented, lamellar structures. The nature and possible pathogenesis of the sea‐blue histiocytosis in this case are discussed briefly. ACTA PATHOL. JPN. 34 : 1
展开▼
