Sea-blue histiocyte syndrome. a secondary degenerative process of macrophages?
Varela-Duran, J.; Roholt, P.C.; Ratliff, N.B.
Archives of Pathology and Laboratory Medicine 104(1): 30-34
1980
ISSN/ISBN: 0003-9985 PMID: 6892519 Document Number: 164272
A case of sea-blue histiocyte syndrome occurred in a 5-year-old boy. Associated laboratory findings include increased levels of hepatic phospholipids and glycosphingolipids, increased serum alkaline phosphatase level, and increased 24-hour urine mucopolysaccharide value. Bone marrow and liver biopsies and excision of chronically enlarged tonsillar tissue were performed. Macrophages that stained sea-blue with Wright-Giemsa stain were found in all tissues. Electron microscopic studies showed degenerating histiocytes packed with abundant loosely arranged myelin figures, some containing fingerprint-like cores formed by concentrically arranged lamellae with a periodicity of 45 A. These ultrastructural findings are compared with those obtained in other reported cases of the sea-blue histiocyte syndrome, as well as with those found in other clinical conditions in which degenerative macrophages are present. We conclude that the sea-blue histiocyte syndrome is a clinical entity that is associated with a variety of disease states; this entity is characterized by the presence of degenerating macrophages in various organs.