L-carnitine deficiency and red blood cell mechanical impairment in beta-thalassemia major.

L-carnitine is an essential element of intermediary metabolism and also was shown to be effective in maintaining normal red blood cell (RBC) function. This study aimed at investigating plasma free L-carnitine concentrations and effectiveness of L-carnitine supplementation in protecting deterioration of RBC properties in beta-thalassemia major patients. Plasma free L-carnitine concentrations were determined in the blood samples obtained before their regular transfusion (about one month after the previous transfusion). Each patient received 100 mg/kg/day oral L-carnitine supplementation. RBC deformability, lipid peroxidation and intracellular free calcium concentrations were investigated before and after this treatment. Plasma free L-carnitine levels and RBC deformability before the treatment were found to be lower whereas lipid peroxidation and intracellular calcium concentration in RBC were higher compared to those of the control subjects before the L-carnitine treatment. After one month supplementation of L-carnitine lipid peroxidation and intracellular calcium concentrations were found to be decreased and RBC deformability was improved, accompanying the significantly increased plasma L-carnitine concentrations. These results suggest that L-carnitine can be used as a supplement in beta-thalassemic patients, to prevent RBC deterioration.