Sea-blue histiocytosis

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Sea-blue histiocytosis
Classification and external resources
ICD-10 D76.3 (ILDS D76.330)

Sea-blue histiocytosis is a cutaneous condition that may occur as a familial inherited syndrome or as an acquired secondary or systemic infiltrative process.[1]:720

Causes[edit]

It can be associated with the gene APOE.[2]

It can also be acquired.[3] Sea-blue histiocyte syndrome is seen in patients receiving fat emulsion as a part of long-term parenteral nutrition (TPN) for intestinal failure.

Pathophysiology and presentation[edit]

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 May-Giemsa[4]/PAS stain hence the name of Sea-Blue Histocyte Syndrome. Sea-blue histiocytosis is also seen in lipid disorders.

See also[edit]

References[edit]

  1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. 
  2. ^ Faivre L, Saugier-Veber P, Pais de Barros JP, et al (November 2005). "Variable expressivity of the clinical and biochemical phenotype associated with the apolipoprotein E p.Leu149del mutation". Eur. J. Hum. Genet. 13 (11): 1186–91. doi:10.1038/sj.ejhg.5201480. PMID 16094309. 
  3. ^ Candoni A, Grimaz S, Doretto P, Fanin R, Falcomer F, Bembi B (October 2001). "Sea-blue histiocytosis secondary to Niemann-Pick disease type B: a case report". Ann. Hematol. 80 (10): 620–2. doi:10.1007/s002770100354. PMID 11732877. 
  4. ^ Suzuki O, Abe M (April 2007). "Secondary sea-blue histiocytosis derived from Niemann-Pick disease" ([dead link]). J Clin Exp Hematop 47 (1): 19–21. doi:10.3960/jslrt.47.19. PMID 17510534.