Sea-Blue Histiocyte Syndrome D012618

Related MeSH Hierarchy (10)

Diseases [C] » Hemic and Lymphatic Diseases [C15] » Lymphatic Diseases » Histiocytosis » Histiocytosis, Non-Langerhans-Cell » Sea-Blue Histiocyte Syndrome

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.   MeSH

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