Das Nandita, Das Chowdhury Tapasi, Chattopadhyay Aindrila, Datta Asoke G
Department of Life Science and Biotechnology, Jadavpur University, Kolkata-700 032, India.
Pol J Pharmacol. 2004 Jan-Feb;56(1):85-96.
The oxidative stress status of the transfusion-dependent Ebeta- and beta-thalassemia patients were studied before and after treatment with vitamin E for a period of four weeks. The level of cellular vitamin antioxidants viz. ascorbic acid and vitamin E in the thalassemia patients were found to be considerably lower compared to normal subjects. The activities of enzymatic antioxidants viz. catalase, glutathione peroxidase and glutathione reductase were found to be drastically reduced in untreated Ebeta- and beta-thalassemic patients when compared to normal subjects. However, the activity of superoxide dis-mutase was found to be increased in both types of untreated thalassemic patients when compared to normal individuals. An increase in superoxide dismutase and a decrease in catalase activity reflects the presence of a severe oxidative stress situation in the erythrocytes of the untreated transfusion dependent Ebeta- and beta-thalassemia patients. Changes in erythrocyte membrane protein pattern in untreated Ebeta- and beta-thalassemia patients when compared to normal erythrocyte further confirm the presence of continued oxidative stress in the ailing thalassemic erythrocytes. All these changes in the antioxidant status as well as the changes in the erythrocyte membrane proteins are ameliorated to considerable extent when the transfusion-dependent Ebeta- and beta-thalassemia patients were treated with vitamin E at a dose of 10 mg/kg/day for a period of four weeks. The patients during the treatment period did not exhibit any side effects and gained in body weight indicating a healthy status. The present study reveals that the lipophilic antioxidant vitamin E could be useful in the management of transfusion-dependant Ebeta- and beta-thalassemia patients.
对依赖输血的Eβ地中海贫血和β地中海贫血患者在接受为期四周的维生素E治疗前后的氧化应激状态进行了研究。发现地中海贫血患者细胞内维生素抗氧化剂（即抗坏血酸和维生素E）的水平与正常受试者相比显著降低。与正常受试者相比，未治疗的Eβ地中海贫血和β地中海贫血患者体内酶促抗氧化剂（即过氧化氢酶、谷胱甘肽过氧化物酶和谷胱甘肽还原酶）的活性大幅降低。然而，与正常个体相比，在两种未治疗的地中海贫血患者中均发现超氧化物歧化酶的活性增加。超氧化物歧化酶增加而过氧化氢酶活性降低反映了未治疗的依赖输血的Eβ地中海贫血和β地中海贫血患者红细胞中存在严重的氧化应激情况。与正常红细胞相比，未治疗的Eβ地中海贫血和β地中海贫血患者红细胞膜蛋白模式的变化进一步证实了患病地中海贫血红细胞中持续存在氧化应激。当依赖输血的Eβ地中海贫血和β地中海贫血患者以10mg/kg/天的剂量接受维生素E治疗四周时，抗氧化状态的所有这些变化以及红细胞膜蛋白的变化在很大程度上得到改善。治疗期间患者未出现任何副作用，体重增加，表明身体状况良好。本研究表明，亲脂性抗氧化剂维生素E可能有助于治疗依赖输血的Eβ地中海贫血和β地中海贫血患者。
