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Aortic features in tangier disease and pathogenetic considerations — Part I. Fatty dots and streaks

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Abstract

Morphological studies of aortic fatty dots and streaks, and the adjacent normally appearing intima, in a 5 3/4-year-old boy who died of pneumonia, showed several hitherto unreported features. In lesions, lipid vacuoles and/or other cytoplasmic “inclusions” (ultrastructurally considered to present complex forms of lipids) were present on occasion in the endothelium but consistently involved the (intimal) smooth muscle cells (SMC). Similar changes were present in the adjacentintima, but were here less prominent and “tapered off” distally. A moderate number of macrophages also contained cytoplasmic lipids but such cells entirely free of lipid inclusions were observed, too.

Most surprisingly, dilated and manycisternae of rough-surfaced endoplasmicreticulum (ER) in the SMC of lesions were associated spatially with cytoplasmic droplets and other forms of lipids.

The results of these studies question the generally accepted central role of macrophages as being primarily involved in the pathogenesis of tissue changes in Tangier disease. It is possible that in view of the absence or paucity of high-density lipoproteins (HDL) and alterations of (their) apo A-I and apo A-II (as well as of other lipids), the arterial SMC may be in some way involved in the metabolism of the above substances in this disorder.

Support of this tentative (and highly speculative) assumption must await further work utilizing tissues and cells other than those containing macrophages and other derivatives of reticuloendothelial system.

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Authors and Affiliations

  1. Departments of Pathology and Paediatrics, The University of Western Ontario, Children's Psychiatric Research Institute, N6A 51C, London, Ontario, Canada

    M. D. Haust

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Presented in part at the 56th Meeting of the European Atherosclerosis Society (European Atherosclerosis Congress)-Santa Margherita di Pula - Cagliari - Italy - October 10–13, 1990.

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Haust, M.D. Aortic features in tangier disease and pathogenetic considerations — Part I. Fatty dots and streaks. Eur J Epidemiol 8 (Suppl 1), 36–47 (1992). https://doi.org/10.1007/BF00145348

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