Sea-Blue Histiocyte Syndrome

**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.

**Tree numbers:** - C10.228.140.163.100.435.825.775 - C15.604.250.410.800 - C16.320.565.189.435.825.775 - C16.320.565.398.641.803.850 - C16.320.565.595.554.825.775 - C18.452.132.100.435.825.775 - C18.452.584.563.641.803.850 - C18.452.648.189.435.825.775 - C18.452.648.398.641.803.850 - C18.452.648.595.554.825.775

**Synonyms:** - Histiocytosis, Sea-Blue - Histiocyte Syndrome, Sea Blue - Sea Blue Histiocytosis - Sea-Blue Histiocyte Disease - Sea-Blue Histiocytosis - Histiocyte Syndromes, Sea Blue