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Concept information

Preferred term

Sea-Blue Histiocyte Syndrome  

Type

  • Topical Descriptor

Entry terms

  • Disease, Sea-Blue Histiocyte
  • Diseases, Sea-Blue Histiocyte
  • Histiocyte Disease, Sea-Blue
  • Histiocyte Diseases, Sea-Blue
  • Histiocyte Syndrome, Sea Blue
  • Histiocyte Syndromes, Sea Blue
  • Histiocytoses, Sea Blue
  • Histiocytoses, Sea-Blue
  • Histiocytosis, Sea Blue
  • Histiocytosis, Sea-Blue
  • Sea Blue Histiocyte Disease
  • Sea-Blue Histiocyte Disease
  • Sea-Blue Histiocyte Diseases
  • Sea Blue Histiocytoses
  • Sea-Blue Histiocytoses
  • Sea Blue Histiocytosis
  • Sea-Blue Histiocytosis

Scope note

  • 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.

History note

  • 1984

In other languages

URI

http://www.yso.fi/onto/mesh/D012618

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