L-carnitine deficiency and red blood cell mechanical impairment in β-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 effective- ness of L-carnitine supplementation in protecting deterioration of RBC properties in β-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 intra- cellular 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 β-thalassemic patients, to prevent RBC deterioration.