Mesh term Sea-Blue Histiocyte Syndrome
Browse to parent terms:Histiocytosis, Non-Langerhans-Cell
Sphingolipidoses
Description
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.Search for this term in our Faculty Database
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