Department of Hematology, the First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, China.
Department of Hematology, the First Affiliated Hospital of Guangxi University of Chinese Medicine, Guangxi, China.
Int J Med Sci. 2019 Jan 1;16(2):302-310. doi: 10.7150/ijms.27829. eCollection 2019.
This study aimed to evaluate the imbalance of erythropoiesis and iron metabolism in patients with thalassemia. 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. 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. 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.
本研究旨在评估铁代谢和红细胞生成失衡在地中海贫血患者中的表现。 2013 年 6 月至 2016 年 12 月期间,在广西医科大学第一附属医院血液科共招募了 192 例非输血依赖型地中海贫血(NTDT)患者、94 例输血依赖型地中海贫血(TDT)患者和 101 名健康对照者。比较各组红细胞生成生物标志物[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)]和铁过载生物标志物[血清铁蛋白(SF)、肝铁浓度(LIC)和心脏 T2*]及铁调素的水平。 地中海贫血患者的 GDF15、EPO、sTfR、LIC 和 SF 水平显著升高。与 NTDT 相比,TDT 患者的 GDF15 和 EPO 水平显著升高。铁过载患者的 EPO、GDF15、SF 和 sTfR 水平明显高于非铁过载患者。与重型β地中海贫血或血红蛋白(Hb)H 病患者相比,中间型β地中海贫血或 HbE/β地中海贫血患者的铁调素水平及铁调素与红细胞生成活性和铁生物标志物水平的比值均较低。NTDT 和 TDT 患者的铁调素水平与 EPO、GDF15 和 sTfR 水平呈负相关,但仅在 TDT 患者中与 SF 和 Hb 水平呈正相关。 地中海贫血患者存在铁过载、铁调素水平降低和无效红细胞生成增加。铁调素与无效红细胞生成的相关性强于铁过载。不同类型地中海贫血之间的红细胞生成和铁代谢失衡存在差异。