Ceroid histiocytosis of the spleen in hyperlipemia: relationship to the syndrome of the sea-blue histiocyte.

Rywlin, Arkadi M., Lopez-Gomez, Alfredo, Tachmes, Pablo, and Pardo, Victoriano: Ceroid histiocytosis of the spleen in hyperlipemia: Relationship to the syndrome of the sea-blue histiocyte. Amer. J. Clin. Path. 56: 572-579, 1971. Morphologic findings in a patient with primary, type 5 hyperlipoproteinemia of Fredrickson and Lees, reported for the first time, include marked splenomegaly with extensive ceroid histiocytosis, fatty metamorphosis and early fibrosis of the liver, and increased numbers of foam cells in the bone marrow. Diseases associated with accumulation of ceroid in various tissues are tabulated. We stress that, with the Wright or Giemsa methods, ceroid granules are stained sea-blue and ceroid-containing macrophages appear as "sea-blue histiocytes." This staining reaction is not pathognomonic for ceroid. The spleen from a patient with "the syndrome of the sea-blue histiocyte" contained numerous ceroid-containing macrophages. In view of the lack of specificity of the "sea-blue histiocyte," it is suggested that, pending its validation, "the syndrome of the sea-blue histiocyte" be renamed "idiopathic ceroid histiocytosis of spleen and marrow" or "Silverstein's syndrome." FREDRICKSON AND LEES 9 have clarified the complex problem of the hyperlipidemias by classifying them into five types on the basis of lipoprotein electrophoresis. Relatively few morphologic data describing the five groups of hyperlipoproteinemias are available. Some of the older reports lack adequate information to assign them to one of the five types. No pathologic findings in patients with clearly established type 5 hyperlipoproteinemia seem to have been recorded. We Received October 15, 1970; revised manuscript received December 8, 1970; accepted for publication December 18, 1970. Address reprint requests to Arkadi M. Rywlin, M.D., Department of Pathology, Mount Sinai Hospital, 4300 Alton Road, Miami Beach, Florida 33140. describe histologic characteristics of the spleen, liver, and bone marrow of a patient with type 5 hyperlipoproteinemia. We stress that ceroid-containing macrophages are identical to sea-blue histiocytes when treated with the Wright or Giemsa stains. The significance of this finding in the evaluation of the recently described syndrome of the sea-blue histiocyte is discussed.