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Lipid membrane peroxidation in beta-thalassemia major
EA Rachmilewitz, SB Shohet and BH Lubin
The composition of membrane lipids was studied in 17 splenectomized and
eight unsplenectomized patients with beta-thalassemia major and compared to
normal controls. The results showed a nearly twofold increase in total cell
lipids; a reduction in the percentage, but not the absolute amount of
phosphatidylethanolamine, and a corresponding increase in
phosphatidylcholine in the lipids; a considerable increase in the
percentage of the saturated fatty acid, palmitic acid, and a reciprocal
decrease in the polyunsaturated fatty acid, arachidonic acid; a twofold
increase in the amount of malonyldialdehyde (MDA) generated after peroxide
threat to the RBC when calculated either per gram hemoglobin or per cell;
no change in the amount of MDA generated when calculated per microgram of
membrane phosphorus at risk per cell; and a considerable decrease in serum
alpha-tocopherol (vitamin E) levels. Thalassemic erythrocytes contain more
lipid per cell which is susceptible to peroxidation. In addition, the
distribution of fatty acids in these cells suggests that autooxidation of
that lipid may have occurred. Autooxidation may be initiated by free
radicals, which are constantly formed in the normal red cell, and may be
especially prevalent when unstable hemoglobins are present. The low MCHC or
some other intracellular defect of thalassemic cells may allow such potent
oxidants to find their way to the cell membrane. Vitamin E, a biologic
antioxidant is decreased in these patients, and clinical supplementation
may be indicated to prevent some of the membrane damage in thalassemia.
Volume 47,
Issue 3,
pp. 495-505,
03/01/1976
Copyright © 1976 by The American Society of Hematology
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