A histochemical study of lipoid pigment storage in chronic granulomatous disease (CGD).
The presence of large pigmented, lipid-laden histiocytes throughout the reticulo-endothelium is a typical feature of CGD. They have been studied histochemically in specimens of spleen, liver and lymph nodes of a 17-year-old female with CGD. The results of such a study indicate that in CGD histiocytes contain a ceroid pigment. The typical autofluorescence of the pigment is related to lipid peroxidation-derived fluorescent compounds, which seem to be directly involved in the PFAS-positivity of ceroid. This suggests that PFAS stain can be considered not only a technique for the demonstration of unsaturated lipids, but also a useful histochemical method in the study of lipid peroxidation. The finding of ceroid storage, which always indicates an excess of free-radical reactions in vivo, is particularly intriguing in CGD, known to be characterized by the failure of a free-radical generating enzymatic system in phagocytes.
D000293:Adolescent; D002566:Ceroid / Q000378:metabolism*; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000378:metabolism* / Q000473:pathology; D006651:Histocytochemistry; D006801:Humans; D050356:Lipid Metabolism; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology; D010860:Pigments, Biological / Q000378:metabolism*; D013154:Spleen / Q000378:metabolism / Q000473:pathology
D002566:Ceroid; D010860:Pigments, Biological