Department of Bio chemistry, NRS Medical College & Hospital, Kolkata, India.
Ann Acad Med Singap. 2010 Jan;39(1):13-6.
Haemoglobin (Hb) E beta-thalassaemia is a common thalassaemic disorder in Southeast Asia and is very common in the eastern and north-eastern parts of India. The disease cause rapid erythrocyte destruction due to the free radical mediated injury but factors for the oxidative injury are not clearly known. We investigated the free reactive iron (non-haem) mediated insult in Hb E beta-thalassaemia.
Thirty Hb E beta-thalassaemic patients (age range, 3 to 15 years) who had undergone blood transfusion at least 1 month prior to sampling and 32 normal healthy individuals (age range, 18 to 30 years) were included in this study. We estimated the ferrozine detected intracellular erythrocytic free reactive iron (nonhaem iron), reduced glutathione (GSH), glutathione reductase activity, cellular damage marker serum thiobarbituric acid reacting substances (TBARS) and also serum ferritin using standard methods.
We found that the erythrocytic free reactive iron was significantly higher (P <0.001) in Hb E beta patients and was about 30% more than in controls. The elevated level of erythrocytic non-haem iron was associated with a high level of serum TBARS which was about 86% higher in patients than in controls. The serum ferritin level was also significantly higher (P <0.001) compared to controls. The erythrocytic reduced glutathione level was significantly lower (P <0.001) at about 65% less in the patients' group and the erythrocytic glutathione reductase enzyme was also found to be significantly lower (P <0.001) in Hb E beta-thalassaemia.
We concluded that a significantly elevated level of erythrocytic free reactive iron and lipid peroxidation end product was associated with low erythrocytic GSH level. This reflects non-haem iron mediated cellular damage in Hb E beta-thalassaemia.
血红蛋白 E 贝塔地贫是东南亚常见的地中海贫血症，在印度东部和东北部非常常见。由于自由基介导的损伤，这种疾病会导致红细胞迅速破坏，但导致氧化损伤的因素尚不清楚。我们研究了血红蛋白 E 贝塔地贫中非血红素介导的自由基损伤。
本研究纳入了 30 名血红蛋白 E 贝塔地贫患者（年龄 3 至 15 岁，均在接受输血至少 1 个月后采血）和 32 名健康对照者（年龄 18 至 30 岁）。我们使用标准方法评估了血菲咯嗪法检测到的细胞内红细胞游离反应性铁（非血红素铁）、还原型谷胱甘肽（GSH）、谷胱甘肽还原酶活性、细胞损伤标志物血清硫代巴比妥酸反应物质（TBARS）以及血清铁蛋白。
我们发现血红蛋白 E 贝塔患者的红细胞游离反应性铁明显升高（P <0.001），比对照组高约 30%。红细胞非血红素铁的升高水平与血清 TBARS 水平升高有关，患者组比对照组高约 86%。血清铁蛋白水平也明显高于对照组（P <0.001）。红细胞还原型谷胱甘肽水平明显降低（P <0.001），患者组比对照组低约 65%，红细胞谷胱甘肽还原酶活性也明显降低（P <0.001）。
我们得出结论，红细胞游离反应性铁和脂质过氧化终产物水平显著升高与红细胞 GSH 水平降低有关。这反映了血红蛋白 E 贝塔地贫中非血红素铁介导的细胞损伤。
