Abstract
Histochemical, biochemical, microfluorometric and electron microscopic studies were made of the spleen of a patient with type I hyperlipoproteinemia. Foam cells were observed that contained a material identified as ceroid on the basis of its autofluorescence, acid-fastness, sudanophilia, PAS-positivity and insolubility in organic solvents. Electron microscopy showed that the ceroid was organized in the form of granules with concentric lamellae of irregular periodicity. The process of formation of these granules is described in detail. The ceroid was considered to represent nondigestible end products of the metabolism of chylomicrons taken up by macrophages in splenic sinusoids.
Full text
PDF





























Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- ABRAMOV A., SCHORR S., WOLMAN M. Generalized xanthomatosis with calcified adrenals. AMA J Dis Child. 1956 Mar;91(3):282–286. doi: 10.1001/archpedi.1956.02060020284010. [DOI] [PubMed] [Google Scholar]
- Burke J. S., Simon G. T. Electron microscopy of the spleen. I. Anatomy and microcirculation. Am J Pathol. 1970 Jan;58(1):127–155. [PMC free article] [PubMed] [Google Scholar]
- Burke J. S., Simon G. T. Electron microscopy of the spleen. II. Phagocytosis of colloidal carbon. Am J Pathol. 1970 Jan;58(1):157–181. [PMC free article] [PubMed] [Google Scholar]
- CIMMINO C. V. CLINICOPATHOLOGICAL CONFERENCE. Va Med Mon (1918) 1965 Jul;92:321–328. [PubMed] [Google Scholar]
- Cheema P. S., Malathi K., Padmanaban G., Sarma P. S. The neurotoxicity of beta-N-oxalyl-L-alphabeta-diaminopropionic acid, the neurotoxin from the pulse Lathyrus sativus. Biochem J. 1969 Mar;112(1):29–33. doi: 10.1042/bj1120029. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Dobbins W. O., 3rd, Rollins E. L. Intestinal mucosal lymphatic permeability: an electron microscopic study of endothelial vesicles and cell junctions. J Ultrastruct Res. 1970 Oct;33(1):29–59. doi: 10.1016/s0022-5320(70)90117-6. [DOI] [PubMed] [Google Scholar]
- Endicott K. M., Lillie R. D. Ceroid, the Pigment of Dietary Cirrhosis of Rats: Its Characteristics and Its Differentiation from Hemofuscin. Am J Pathol. 1944 Jan;20(1):149–153. [PMC free article] [PubMed] [Google Scholar]
- FISHER E. R., HELLSTROM H. R. CEROID-LIKE COLONIC HISTIOCYTOSIS. Am J Clin Pathol. 1964 Dec;42:581–593. [PubMed] [Google Scholar]
- FREDRICKSON D. S., ONO K., DAVIS L. L. LIPOLYTIC ACTIVITY OF POST-HEPARIN PLASMA IN HYPERGLYCERIDEMIA. J Lipid Res. 1963 Jan;4:24–33. [PubMed] [Google Scholar]
- FREEMAN J. A., SPURLOCK B. O. A new epoxy embedment for electron microscopy. J Cell Biol. 1962 Jun;13:437–443. doi: 10.1083/jcb.13.3.437. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Ferrans V. J., Hibbs R. G., Buja L. M. Nucleoside phosphatase activity in atrial and ventricular myocardium of the rat: a light and electron microscopic study. Am J Anat. 1969 May;125(1):47–85. doi: 10.1002/aja.1001250104. [DOI] [PubMed] [Google Scholar]
- Fredrickson D. S. Classification and features of the lipidoses affecting the nervous system. Pathol Eur. 1968;3(2):121–142. [PubMed] [Google Scholar]
- Fredrickson D. S., Levy R. I., Lees R. S. Fat transport in lipoproteins--an integrated approach to mechanisms and disorders. N Engl J Med. 1967 Feb 2;276(5):273–concl. doi: 10.1056/NEJM196702022760507. [DOI] [PubMed] [Google Scholar]
- Greten H., Levy R. I., Fredrickson D. S. Evidence for separate monoglyceride hydrolase and triglyceride lipase in post-heparin human plasma. J Lipid Res. 1969 May;10(3):326–330. [PubMed] [Google Scholar]
- Györkey F., Shimamura T., O'Neal R. M. The fine structure of ceroid in human atheroma. J Histochem Cytochem. 1967 Dec;15(12):732–736. doi: 10.1177/15.12.732. [DOI] [PubMed] [Google Scholar]
- HERS H. G. INBORN LYSOSOMAL DISEASES. Gastroenterology. 1965 May;48:625–633. [PubMed] [Google Scholar]
- Hartroft W. S., Porta E. A. Ceroid. Am J Med Sci. 1965 Sep;250(3):324–345. [PubMed] [Google Scholar]
- Hibbs R. G., Ferrans V. J., Cipriano P. R., Tardiff K. J. A histochemical and electron microscopic study of Gaucher cells. Arch Pathol. 1970 Feb;89(2):137–153. [PubMed] [Google Scholar]
- Hoffman H. N., Fredrickson D. S. Tangier disease (familial high density lipoprotein deficiency). Clinical and genetic features in two adults. Am J Med. 1965 Oct;39(4):582–593. doi: 10.1016/0002-9343(65)90081-1. [DOI] [PubMed] [Google Scholar]
- Kwiterovich P. O., Jr, Sloan H. R., Fredrickson D. S. Glycolipids and other lipid constituents of normal human liver. J Lipid Res. 1970 Jul;11(4):322–330. [PubMed] [Google Scholar]
- LANDING B. H., SHIRKEY H. S. A syndrome of recurrent infection and infiltration of viscera by pigmented lipid histiocytes. Pediatrics. 1957 Sep;20(3):431–438. [PubMed] [Google Scholar]
- Lough J., Fawcett J., Wiegensberg B. Wolman's disease. An electron microscopic, histochemical, and biochemical study. Arch Pathol. 1970 Feb;89(2):103–110. [PubMed] [Google Scholar]
- MOORE R. D., MUMAW V. R., SCHOENBERG M. D. THE STRUCTURE OF THE SPLEEN AND ITS FUNCTIONAL IMPLICATIONS. Exp Mol Pathol. 1964 Feb;33:31–50. doi: 10.1016/0014-4800(64)90016-4. [DOI] [PubMed] [Google Scholar]
- NEGLIA W., BURROWS L., THOMPSON S. W., 2nd, SCHAFFNER F. Ultrastructural studies of hepatic pigment following administration of intravenous fat. Lab Invest. 1963 Mar;12:378–385. [PubMed] [Google Scholar]
- NOVIKOFF A. B., GOLDFISCHER S. Nucleosidediphosphatase activity in the Golgi apparatus and its usefulness for cytological studies. Proc Natl Acad Sci U S A. 1961 Jun 15;47:802–810. doi: 10.1073/pnas.47.6.802. [DOI] [PMC free article] [PubMed] [Google Scholar]
- OPPENHEIMER E. H., ANDREWS E. C., Jr Ceroid storage disease in childhood. Pediatrics. 1959 Jun;23(6):1091–1102. [PubMed] [Google Scholar]
- Parker F., Odland G. F. Electron microscopic similarities between experimental xanthomas and human eruptive xanthomas. J Invest Dermatol. 1969 Feb;52(2):136–147. [PubMed] [Google Scholar]
- Patrick A. D., Lake B. D. Deficiency of an acid lipase in Wolman's disease. Nature. 1969 Jun 14;222(5198):1067–1068. doi: 10.1038/2221067a0. [DOI] [PubMed] [Google Scholar]
- ROBERTS D. K., LATTA J. S. ELECTRON MICROSCOPIC STUDIES ON THE RED PULP OF THE RABBIT SPLEEN. Anat Rec. 1964 Jan;148:81–101. doi: 10.1002/ar.1091480108. [DOI] [PubMed] [Google Scholar]
- Roberts W. C., Levy R. I., Fredrickson D. S. Hyperlipoproteinemia. A review of the five types with first report of necropsy findings in type 3. Arch Pathol. 1970 Jul;90(1):46–56. [PubMed] [Google Scholar]
- Rostgaard J., Behnke O. Fine structural localization of adenine nucleoside phosphatase activity in the sarcoplasmic reticulum and the T system of rat myocardium. J Ultrastruct Res. 1965 Jun;12(5):579–591. doi: 10.1016/s0022-5320(65)80049-1. [DOI] [PubMed] [Google Scholar]
- Schneck L., Volk B. W., Saifer A. The gangliosidoses. Am J Med. 1969 Feb;46(2):245–263. doi: 10.1016/0002-9343(69)90009-6. [DOI] [PubMed] [Google Scholar]
- Simon G. T., Burke J. S. Electron microscopy of the spleen. 3. Erythro-leukophagocytosis. Am J Pathol. 1970 Mar;58(3):451–470. [PMC free article] [PubMed] [Google Scholar]
- Suzuki K., Chen G. C. GM1-gangliosidosis (gneralized gangliosidosis). Morphology and chemical pathology. Pathol Eur. 1968;3(2):389–408. [PubMed] [Google Scholar]
- TANAKA Y., BRECHER G., FREDRICKSON D. S. [Cells in Nieman-Pick disease and various other lopoidoses]. Nouv Rev Fr Hematol. 1963 Jan-Feb;3:5–16. [PubMed] [Google Scholar]
- TERRY R. D., SPERRY W. M., BRODOFF B. Adult lipidosis resembling Niemann-Pick's disease. Am J Pathol. 1954 Mar-Apr;30(2):263–285. [PMC free article] [PubMed] [Google Scholar]
- TORO I., RUZSA P., ROHLICH P. Ultrastructure of early phagocytic stages in sinus endothelial and Kupffer cells of the liver. Exp Cell Res. 1962 Mar;26:601–603. doi: 10.1016/0014-4827(62)90168-4. [DOI] [PubMed] [Google Scholar]
- Volk B. W., Wallace B. J., Aronson S. M. Some ultrastructural and histochemical aspects of lipidoses. Pathol Eur. 1968;3(2):200–217. [PubMed] [Google Scholar]
- Warren S., Root H. F. Lipoid-Containing Cells in the Spleen in Diabetes with Lipemia. Am J Pathol. 1926 Jan;2(1):69–80.3. [PMC free article] [PubMed] [Google Scholar]
- Winkler H. H., Frame B., Saeed S. M., Spindler A. C., Brouillette J. N. Ceroid storage disease, complicated by rupture of the spleen. Am J Med. 1969 Feb;46(2):297–301. doi: 10.1016/0002-9343(69)90013-8. [DOI] [PubMed] [Google Scholar]
- Yamori T., Mori Y. Fine structure of the reticuloendothelial system in the spleen with special reference to the red pulp. Tohoku J Exp Med. 1967 Apr;91(4):367–374. doi: 10.1620/tjem.91.367. [DOI] [PubMed] [Google Scholar]





























