Sea-blue histiocyte syndrome

Sea-blue histiocyte syndrome

Sea-blue histiocyte syndrome is a sphingolipid disorder (a sphingolipidosis).

It can be associated with the gene APOE

Sea-blue histiocyte syndrome is seen in patients receiving fat emulsion as a part of long-term parental nutrition (TPN) for intestinal failure. The high lipid content in the blood leads to excessive cytoplasm loading of lipids within histiocytes. The subsequent incomplete degradation of these lipids leads to the formation of cytoplasmic lipid pigments. High lipid content may also cause membrane abnormality of the hemopoietic cells which is recognized by macrophages and therefore, increased accumulation within the bone marrow. These lipid laden histiocytes appear blue with Giemsa/PAS stain hence the name of Sea-Blue Histocyte Syndrome. Sea-blue histiocytosis is also seen in lipid disorders.

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