Chronic granulomatous disease of childhood and sea-blue histiocytosis. A pathologic study of an autopsy case.
A boy, who started remittent fever reaching 40 degrees 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.
D001344:Autopsy; D002648:Child; D006026:Glycoside Hydrolases / Q000097:blood; D006105:Granulomatous Disease, Chronic / Q000097:blood / Q000150:complications / Q000473:pathology*; D006801:Humans; D007962:Leukocytes / Q000201:enzymology; D008055:Lipids / Q000097:blood; D008175:Lung Neoplasms / Q000097:blood / Q000150:complications / Q000473:pathology*; D008206:Lymphatic Diseases / Q000097:blood / Q000150:complications / Q000473:pathology*; D008247:Lysosomes / Q000201:enzymology; D008297:Male; D008854:Microscopy, Electron
D008055:Lipids; D006026:Glycoside Hydrolases