Erythrocyte labile iron pool indicating concealed iron overload in non-transfusion-dependent β-thalassemia

Boonyanuch Chutvanichkul, P. Vattanaviboon, Sumana Mas-oodi, Y. U-pratya, Wanchai Wanachiwanawin
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Abstract

Abstract Objectives Not only do transfusion-dependent thalassemia (TDT) patients have a risk of clinical consequences arising from iron overload, non-transfusion-dependent thalassemia (NTDT) patients may encounter it also. In this study, we demonstrated the usefulness of intra-erythrocyte labile iron pool (LIP) measurement in NTDT patients with HbE/β-thalassemia in revealing a concealed iron overload and the resultant oxidative cell damage. Methods LIP and ferritin levels were assayed in 20 HbE/β-thalassemia patients, comprising 10 NTDT and 10 TDT patients and 10 healthy individuals. Erythrocyte oxidative stress parameters (intraerythrocyte reactive oxygen species (ROS) levels, numbers of erythrocyte vesicles, and cell apoptosis) were also determined and their correlations to LIP and ferritin levels were analyzed. Results LIP levels (based on different mean fluorescence intensity (MFI) values obtained by flow cytometry) were high in both the NTDT (54 [42–90]) and TDT (71 [68–77]) patients compared to the healthy individuals (43 [31–61]). The ferritin level observed in the NTDT group (2,149 [781–4,071] ng/mL) was lower than that of the TDT group (4,885 [2,343–7,826] ng/mL). The LIP level was significantly correlated with the NTDT patients’ age and all oxidative stress parameters, but the ferritin level was not. Correlation analysis predicted that NTDT patients without receiving any therapy aged 32.5 years old or over are likely at risk of iron overload status and oxidative cell damage. Conclusions The results indicate that LIP may be a helpful alternative parameter for defining the risk of iron overload.
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红细胞可溶性铁库显示非输血依赖型β地中海贫血症患者隐性铁超载
摘要 目的 不仅输血依赖型地中海贫血(TDT)患者有可能因铁超载而导致临床后果,非输血依赖型地中海贫血(NTDT)患者也可能遇到这种情况。在这项研究中,我们证明了在 HbE/β 地中海贫血症的 NTDT 患者中进行红细胞内易变铁池(LIP)测量对揭示隐性铁超载及由此导致的细胞氧化损伤非常有用。方法 对 20 名 HbE/β 地中海贫血症患者(包括 10 名 NTDT 和 10 名 TDT 患者)和 10 名健康人进行 LIP 和铁蛋白水平检测。还测定了红细胞氧化应激参数(红细胞内活性氧(ROS)水平、红细胞囊泡数量和细胞凋亡),并分析了它们与 LIP 和铁蛋白水平的相关性。结果 与健康人(43 [31-61])相比,NTDT(54 [42-90])和 TDT(71 [68-77])患者的 LIP 水平(基于流式细胞术获得的不同平均荧光强度 (MFI) 值)都很高。在 NTDT 组中观察到的铁蛋白水平(2,149 [781-4,071] 纳克/毫升)低于 TDT 组(4,885 [2,343-7,826] 纳克/毫升)。LIP 水平与 NTDT 患者的年龄和所有氧化应激参数有明显相关性,但与铁蛋白水平无明显相关性。相关性分析预测,32.5 岁或以上未接受任何治疗的 NTDT 患者很可能面临铁超载状态和氧化细胞损伤的风险。结论 结果表明,LIP 可能是界定铁超载风险的一个有用的替代参数。
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