Sea-Blue Histiocyte Syndrome (definition)

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Sea-Blue Histiocyte Syndrome

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Definition: A congenital disease caused by an inborn error involving APOLIPOPROTEINS E leading to abnormal LIPID METABOLISM and the accumulation of GLYCOSPHINGOLIPIDS, particularly SPHINGOMYELINS in the HISTIOCYTES. This disorder is characterized by SPLENOMEGALY and the sea-blue histiocytes in the spleen and bone marrow after May Grunwald staining.

See Also Niemann-Pick Diseases

Other names Histiocytosis, Sea-Blue; Syndromes, Sea-Blue Histiocyte; Syndrome, Sea-Blue Histiocyte; Sea-Blue Histiocytosis; Sea-Blue Histiocytoses; Sea-Blue Histiocyte Syndromes; Sea-Blue Histiocyte Diseases; Sea Blue Histiocytoses; Sea Blue Histiocyte Syndrome; Sea Blue Histiocyte Disease; Histiocytosis, Sea Blue; Histiocytoses, Sea-Blue; Histiocytoses, Sea Blue; Histiocyte Syndromes, Sea-Blue; Histiocyte Syndrome, Sea-Blue; Histiocyte Diseases, Sea-Blue; Histiocyte Disease, Sea-Blue; Diseases, Sea-Blue Histiocyte; Disease, Sea-Blue Histiocyte; Sea-Blue Histiocyte Disease

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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