Fatty-acid peroxidation in relation to trace elements in serum of patients with homozygous sickle-cell anemia and beta-thalassemia: a gas-chromatographic study.

Due mainly to a deeply disturbed iron metabolism, intense production of oxygen-derived free radicals occurs in genetic hemoglobinopathies such as homozygous sickle-cell anemia and beta-thalassemia. Together with impairments in the natural factors involved in oxy-radical detoxication, this results in intense oxidative stress leading to lipid peroxidation in the blood components. In search of peroxidation effects, we undertook a gas chromatographic study of both the total and phospholipid-bound fatty acids in the serum from sickle-cell disease and beta-thalassemia patients. Specific alterations of pathologic origin have been evidenced in the profiles of total and phospholipidic fatty acids, as well as in the elongation-desaturation ratios of the total fatty acids. Results are consistent with lipid peroxidations and fatty-acid biosynthesis disturbances in both diseases, but more severe in thalassemia than in sickle-cell anemia. Increased serum selenium in the latter disease might exert a protective action against lipid peroxidation.