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The Korean Journal of Pathology 1982;16(3): 540-544.
Ceroid 조직구증 1예
A Case of the Sea-Blue Histiocyte Syndrome
Sea-Blue Histiocyte syndrome is one of the lipidosis which has characterize large reticuloendothelial cell in spleen, liver, bone marrow and lymph node, and shows splenomegaly. This large reticuloendothelial cell so called Sea-Blue Histiocyte is a large, up to 20μ in diameter. In some cells, the cytoplasm was packed with large, homogeneous, and blue-staining granules that were 3-4μ in diameter. The Sea-Blue Histiocytosis has been associated with abnormal lipid metabolism and the abnormal substance in the Sea-Blue Histiocyte is though to be Ceroid. Ceroid is result from the oxidation and polymerization of unsaturated lipids. The Sea-Blue Histiocyte syndrome has been reported both as a primary disorder and in association with variety of disease. Recently we experienced a case of Sea-Blue Histiocyte Syndrome with nephrotic syndrome and hepatopathy. Bone marrow aspiration and liver biopsy showed many Sea-Blue Histocyte. The patient was died but we can't take autopsy.
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