Apoptosis in Thalassemia major Reduced by a Butyrate Derivative
El-Beshlawy, A.; Seoud, H.; S. IBRAHIM, Ahmed; Youssry, I.; Gabre, H.; Isma’eel, H.; Aoun, E.; Taher, A.;
Abstract
Background and Objectives: In cases of β-thalassemia major, apoptosis appears to be greatly enhanced in the early-stage erythroid precursors in the bone marrow leading to ineffective erythropoiesis. L-Carnitine is found to strongly reduce apoptosis in different diseases. We investigated the effect of oral L-carnitine therapy on apoptosis in thalassemia major patients. Methods: Eighteen thalassemia major patients with a mean age of 12.2 ± 6.6 years were included. Detection of apoptosis was done by photometric enzyme immunoassay (ELISA) and agarose gel electrophoresis before and after 6 months of oral therapy with L-carnitine (50 mg/kg/day). Results: A significant decrease of apoptosis frequency in the erythroid precursors in the bone marrow of studied cases was noted after therapy. The quantity of nucleosomes measured by ELISA dropped from 3.65 ± 1.338 to 1.60 ± 0.65 after therapy (p = 0.005). A positive ladder pattern reflecting apoptosis on agarose gel electrophoresis was detected in 88.9% of cases prior to treatment versus 16.7% after therapy (p = 0.006). Patients also had a significant decrease in the frequency of transfusions and increase in the pre-transfusion hemoglobin levels after therapy. Conclusion: L-Carnitine seems to be a good modulator of apoptotic processes in thalassemic patients leading to a decreased frequency of programmed erythroblast death and general improvement of the disease condition. Copyright © 2005 S. Karger AG.
Other data
Title | Apoptosis in Thalassemia major Reduced by a Butyrate Derivative | Authors | El-Beshlawy, A. ; Seoud, H. ; S. IBRAHIM, Ahmed ; Youssry, I. ; Gabre, H. ; Isma’eel, H. ; Aoun, E. ; Taher, A. | Issue Date | 2005 | Publisher | KARGER | Journal | Acta Haematologica | DOI | 3 155 https://api.elsevier.com/content/abstract/scopus_id/27544462407 114 10.1159/000087890 |
PubMed ID | 114 | Scopus ID | 2-s2.0-27544462407 | Web of science ID | WOS:000232664900006 |
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