Sea-blue histiocytosis is a cutaneous condition that may occur as a familial inherited syndrome or as an acquired secondary or systemic infiltrative process.
Causes
It can be associated with the gene
APOE
Apolipoprotein E (APOE) is a protein involved in the metabolism of fats in the body of mammals. A subtype is implicated in Alzheimer's disease and cardiovascular disease.
APOE belongs to a family of fat-binding proteins called apolipoproteins. ...
.
It can also be acquired.
Sea-blue histiocyte syndrome is seen in patients receiving fat emulsion as a part of long-term
parenteral nutrition
Parenteral nutrition (PN) is the feeding of nutritional products to a person intravenously, bypassing the usual process of eating and digestion. The products are made by pharmaceutical compounding companies. The person receives a nutritional mix ...
(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
Giemsa stain (), named after German chemist and bacteriologist Gustav Giemsa, is a nucleic acid stain used in cytogenetics and for the histopathological diagnosis of malaria and other parasites.
Uses
It is specific for the phosphate groups of ...
[ ]/PAS stain hence the name of Sea-Blue Histocyte Syndrome.
Sea-blue histiocytosis is also seen in lipid disorders.
Diagnosis
See also
*
Non-X histiocytosis
Non-X histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of monocytes/macrophages, as opposed to X-type histiocytoses in which the infiltrates contain Langerhans cells. Conditions included in this ...
References
External links
{{Histiocytosis
Monocyte- and macrophage-related cutaneous conditions