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Sea-Blue Histiocyte Syndrome  

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Tipo

  • mesh:Descriptor

Definición

  • 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.

Etiquetas alternativas

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

En otras lenguas

URI

http://data.loterre.fr/ark:/67375/JVR-D7JTHJPX-V

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