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KMID : 0357919820160030540
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Korean Journal of Pathology
1982 Volume.16 No. 3 p.540 ~ p.544
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A Case of the Sea-Bule Histiocyte Syndrome
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서헌석/Hun Suk Suh
정용호/정화순/최일영/기춘석/김춘원/박승함/김기홍/Young Ho Chung/Wha Soon Chung/Il Young Choi/Choon Suk Ki/Choon Won Kim/Seung Hahm Park/Ki Hong Kim
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Abstract
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Sea-Bule Histiocyte syndrome is one of the lipidosis which has characterized large
reticuloendothelial cell in spleen, liver, tone marrow and lymph node, and shows
splenomegaly. This large reticuloendothelial cell so called Sea-Blue Histiocyte is a large,
up to 20 y in diameter. In some cells, the cytoplasm was packed with large,
homogeneous, and blue-staining granules that were 3∼4 μ 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 histiocyte. The patient was died tut we can't take autopsy.
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