Sea-blue histiocytosis explained

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]

Causes

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

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

• Non-X histiocytosis

Notes and References

1. Book: James, William D. . Berger, Timothy G.. Andrews' Diseases of the Skin: clinical Dermatology . Saunders Elsevier . 2006 . 978-0-7216-2921-6 . etal.
2. Faivre L, Saugier-Veber P, Pais de Barros JP, etal . Variable expressivity of the clinical and biochemical phenotype associated with the apolipoprotein E p.Leu149del mutation . Eur. J. Hum. Genet. . 13 . 11 . 1186–91 . November 2005 . 16094309 . 10.1038/sj.ejhg.5201480. free .
3. Candoni A, Grimaz S, Doretto P, Fanin R, Falcomer F, Bembi B . Sea-blue histiocytosis secondary to Niemann-Pick disease type B: a case report . Ann. Hematol. . 80 . 10 . 620–2 . October 2001 . 11732877 . 10.1007/s002770100354.
4. Suzuki O, Abe M . Secondary sea-blue histiocytosis derived from Niemann-Pick disease . J Clin Exp Hematop . 47 . 1 . 19–21 . April 2007 . 17510534 . 10.3960/jslrt.47.19. free .