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Imbalance of erythropoiesis and iron metabolism in patients with thalassemia

机译:地中海贫血患者的红细胞生成和铁代谢失衡

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摘要

>Aim: This study aimed to evaluate the imbalance of erythropoiesis and iron metabolism in patients with thalassemia.>Methods: 192 patients with non-transfusion-dependent thalassemia (NTDT), 94 patients with transfusion-dependent thalassemia (TDT) and 101 healthy controls were recruited between June 2013 and December 2016 in the Hematology Department, the First Affiliated Hospital of Guangxi Medical University. The groups were compared in terms of levels of erythropoiesis biomarkers [growth differentiation factor 15 (GDF15), erythropoietin (EPO) and soluble transferrin receptor (sTfR)] and of iron overload biomarkers [serum ferritin (SF), liver iron concentration (LIC) and cardiac T2*] and hepcidin.>Results: The levels of GDF15, EPO, sTfR, LIC and SF were significantly higher in patients with thalassemia. The levels of GDF15 and EPO were significantly higher in patients with TDT compared to NTDT. Those with iron overload had higher EPO, GDF15, SF and sTfR levels compared with non-iron overload patients. Hepcidin levels and ratios of hepcidin to erythropoietic activity and to iron biomarker levels were lower in patients with β-thalassemia intermedia or hemoglobin (Hb) E/β-thalassemia than in patients with HbH disease. The hepcidin levels were correlated negatively with the levels of EPO, GDF15 and sTfR in patients with NTDT and TDT, but correlated positively with SF and Hb levels only in patients with TDT.>Conclusions: Patients with thalassemia showed iron overload, reduced hepcidin levels, and a greater extent of ineffective erythropoiesis. The hepcidin levels were more strongly related to ineffective erythropoiesis compared with iron overload. The imbalance between erythropoiesis and iron metabolism differed across different thalassemia types.
机译:>目的:该研究旨在评估地中海贫血患者的红细胞生成和铁代谢失衡。>方法: 192例非输血依赖性地中海贫血(NTDT)患者,94例于2013年6月至2016年12月在广西医科大学第一附属医院血液科招募了101名健康对照者,这些患者具有输血依赖性地中海贫血(TDT)和101名健康对照。比较各组的红细胞生成生物标志物水平[生长分化因子15(GDF15),促红细胞生成素(EPO)和可溶性转铁蛋白受体(sTfR)]和铁超载生物标志物[血清铁蛋白(SF),肝铁浓度(LIC)] >结果:地中海贫血患者的GDF15,EPO,sTfR,LIC和SF的水平明显升高。与NTDT相比,TDT患者中GDF15和EPO的水平明显更高。与非铁超负荷患者相比,铁超负荷患者的EPO,GDF15,SF和sTfR水平更高。中度地中海贫血或血红蛋白(Hb)E /β地中海贫血患者的铁调素水平,铁调素与促红细胞生成活性和铁生物标志物的比率均低于HbH疾病患者。 NTDT和TDT患者中铁调素水平与EPO,GDF15和sTfR呈负相关,而仅TDT患者中铁调素与SF和Hb水平呈正相关。>结论:地中海贫血患者显示铁超负荷,铁调素水平降低和更大范围的无效红细胞生成。与铁超负荷相比,铁调素水平与无效的红细胞生成更密切相关。不同类型的地中海贫血,红细胞生成和铁代谢之间的不平衡有所不同。

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