Tsamesidis Ioannis, Fozza Claudio, Vagdatli Eleni, Kalpaka Anastasia, Cirotto Carla, Pau Maria Carmina, Pantaleo Antonella, Turrini Francesco, Grigoriou Elisavet, Lymperaki Eugenia
Department of Medicine, Section of Internal Medicine, University of Verona, Italy.
Department of Biomedical Sciences, University of Sassari, Italy.
Adv Clin Exp Med. 2017 Aug;26(5):789-793. doi: 10.17219/acem/63746.
Beta thalassemia major (BT) is an inherited blood disorder caused by reduced or absent synthesis of the hemoglobin beta chains, associated with profound anemia, jaundice, splenomegaly, expanded bone marrow volume, siderosis and cardiomegaly. Because of repeated blood transfusions, BT patients are subjected to peroxidative tissue injury due to secondary iron overload.
The aim of the study was to analyze: 1) the total antioxidant capacity (TAC) value in BT patients (study group) and their healthy controls (control group) from Greece (Central Macedonia) and Italy (Sardinia); correlations between 2) the TAC and ferritin levels of BT patients, and 3) the TAC and ferritin values in BT patients with different chelation therapies.
The studied group consisted of 60 subjects diagnosed with BT (41 female, mean age: 41.5 ± 9.5 years) and 40 healthy controls matched with age and sex (31 female, mean age: 38.5 ± 3.7 years). Desferrioxamine (DFO) was the basic previous chelation regimen for all BT patients. Antioxidant activity was assayed spectrophotometrically, using a TAC Kit (Total Antioxidant Capacity Colorimetric assay kit, produced by Cayman Chemical Co.), and ferritin was assayed by immunoturbidimetry.
Lower levels of TAC were observed in BT patients of both countries when compared with controls (1.83 mmol/L vs 2.7 mmol/L in the Italian study group and controls and 2.42 mmol/L vs 3.2 mmol/L in the Greek study group and controls). There were no significant correlations between plasmatic TAC and ferritin. Furthermore, deferasirox was the only chelation treatment in which TAC showed a correlation in both regions.
Our results potentially suggest that the reduced levels of TAC detectable in BT patients could demonstrate their reduced antioxidant defensive mechanisms.
重型β地中海贫血（BT）是一种遗传性血液疾病，由血红蛋白β链合成减少或缺乏引起，伴有严重贫血、黄疸、脾肿大、骨髓体积增大、铁沉着症和心脏肥大。由于反复输血，BT患者因继发性铁过载而遭受过氧化组织损伤。
本研究的目的是分析：1）来自希腊（中马其顿）和意大利（撒丁岛）的BT患者（研究组）及其健康对照（对照组）的总抗氧化能力（TAC）值；2）BT患者的TAC与铁蛋白水平之间的相关性；3）接受不同螯合疗法的BT患者的TAC与铁蛋白值。
研究组由60名诊断为BT的受试者（41名女性，平均年龄：41.5±9.5岁）和40名年龄和性别匹配的健康对照（31名女性，平均年龄：38.5±3.7岁）组成。去铁胺（DFO）是所有BT患者先前的基本螯合方案。使用TAC试剂盒（由开曼化学公司生产的总抗氧化能力比色测定试剂盒）通过分光光度法测定抗氧化活性，并通过免疫比浊法测定铁蛋白。
与对照组相比，两个国家的BT患者的TAC水平均较低（意大利研究组和对照组分别为1.83 mmol/L对2.7 mmol/L，希腊研究组和对照组分别为2.42 mmol/L对3.2 mmol/L）。血浆TAC与铁蛋白之间无显著相关性。此外，地拉罗司是唯一一种在两个地区TAC均显示出相关性的螯合治疗。
我们的结果可能表明，在BT患者中可检测到的TAC水平降低可能表明其抗氧化防御机制减弱。
