Sea-blue histiocytosis associated with hyperlipidaemia.

Abstract

A patient with sea-blue histiocytosis in spleen and bone marrow with an accompanying hyperlipidaemia is described. The hyperlipidaemia was due to an increase in "free" cholesterol, lecithin, and triglycerides. Despite these findings lecithin-cholesterol acyl transferase activity was normal. Although the precise biochemical defect was not identified, there was a failure of transport of cholesterol from chylomicrons in vitro. We propose that the sea-blue histiocyte is a marker, in some cases, of abnormal lipid metabolism.

Acquired lipidosis of marrow macrophages: birefringent blue crystals and Gaucher-like cells, sea-blue histiocytes, and grey-green crystals.

“…In two marrows studied we were unable to find the 'fingerprint' pattern inclusions described by Parker et al (1976) resembling the appearance seen in Niemann-Pick disease.…”

Sea blue histiocytosis in a patient with chronic non-neuropathic Niemann-Pick disease.

“…In the second group, histiocytes are confined to the spleen and bone marrow, and other diseases frequently co-exist (Silverstein and Ellefson, 1972). It is likely that in some cases the sea blue histiocyte is a macrophage which has ingested large amounts of particulate lipid in the process of clearing such particles from the blood and tissues and cannot digest them (Parker et al, 1976). Though the precise biochemical defect cannot always be identified it seems reasonable to suggest that in most instances a structurally defective or partially or completely absent enzyme leads to an accumulation of its natural substrate within the cell, producing a cell morphologically similar to the sea blue histiocyte.…”

Splenic Foam Cells: A Clinico-Pathologic Analysis of 92 Splenectomized Patients

“…Splenic foam cells were previously reported to be associated with a variety of conditions including the storage diseases of Gaucher's and Niemann-Pick [1,2], (3-thalassemia major (THAL) [3,4], hyperlipemia [5,6], chronic granulocytic leukemia (CGL) [7][8][9], idi opathic thrombocytopenic purpura (ITP) [10][11][12][13][14][15][16][17][18][19] and congenital dyserythropoietic anemia, HEM PAS [20]. The nomenclature used for these cells as well as the nature of the substance within them have been a mat ter of controversy over the years.…”

“…The nomenclature used for these cells as well as the nature of the substance within them have been a mat ter of controversy over the years. Terminology such as 'sea-blue histiocytosis' [5,6,[21][22][23][24][25][26], Gaucher and Gaucher-like cells [4,[7][8][9]20] and splenic lipidosis [27] have been repeatedly used as synonyms for these cells.…”