Evaluation of the levels of the markers of ineffective erythropoiesis (transforming growth factor-beta, growth differentiation factor 15 and erythropoietin) in patient with ß-thalassemia syndrome and its correlation to clinical and hematological parameters

IF 0.1 Q4 HEMATOLOGY
M. Yousif, H. Al-Mamoori
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引用次数: 0

Abstract

BACKGROUND: Anemia of β thalassemia results from a combination of ineffective erythropoiesis and hemolysis. This stimulates erythropoietin (EPO) production, which causes expansion of the bone marrow and may lead to serious deformities of the skull and long bones. Ineffective erythropoiesis also induces the release of growth differentiation factor 15 (GDF-15) and transforming growth factor-beta (TGF-β) which have been identified as regulators of hepcidin expression. OBJECTIVE: The objective is to evaluate the level of TGF-β, GDF-15, and EPO in patients with thalassemia syndrome. PATIENTS, MATERIALS AND METHODS: Patient samples were collected from Thalassemia Center of Ibn Al-Baladi Hospital. This study included 35 patients with thalassemia, 18 patients with beta-thalassemia major and 17 patients with beta-thalassemia intermedia. The age of studied group was 3–17 years. Twenty control healthy subjects were included for comparison who were age- and sex-matched with the patients group. Gel tube was used for collection of serum for enzyme-linked immunosorbent assay test for GDF-15, TGF-β, and EPO). RESULTS: There was a highly significant difference in GDF-15 and EPO levels among studied groups (P < 0.001). In addition, there was no significant difference in TGF-β level among studied groups (P > 0.05). TGF-β, GDF-15, and EPO were not significantly correlated to splenomegaly, hepatosplenomegaly, and frequency of blood transfusion duration in patients with beta-thalassemia major (P > 0.05), while TGF-β and EPO were significantly correlated to splenomegaly, hepatosplenomegaly in patients with beta-thalassemia intermedia but GDF-15 was not significantly correlated. In patients with beta-thalassemia major, EPO was negatively correlated to hemoglobin, packed cell volume, mean corpuscular volume, and red blood cells (RBC) count whereas GDF-15 significantly correlated to lymphocyte and neutrophil counts. TGF-β was significantly correlated to platelet count. In patients with beta-thalassemia intermedia, EPO and GDF-15 were not correlated to any hematological parameters whereas TGF-β was significantly correlated to RBC counts. CONCLUSION: Marker of erythropoiesis GDF-15, EPO was highly expressed in patient with beta-thalassemia major and beta-thalassemia intermedia as compared to the control group and this can be used as a future therapeutic goal for the suppression of ineffective erythropoiesis.
ß-地中海贫血综合征患者无效红细胞生成标志物(转化生长因子- β、生长分化因子15和促红细胞生成素)水平的评价及其与临床和血液学参数的相关性
背景:β地中海贫血是红细胞生成无效和溶血综合作用的结果。这会刺激红细胞生成素(EPO)的产生,从而导致骨髓膨胀,并可能导致颅骨和长骨的严重畸形。无效的红细胞生成也会诱导生长分化因子15(GDF-15)和转化生长因子β(TGF-β)的释放,这两种因子已被确定为铁调素表达的调节因子。目的:评估地中海贫血综合征患者TGF-β、GDF-15和EPO的水平。患者、材料和方法:患者样本取自伊本·巴拉迪医院地中海贫血中心。这项研究包括35名地中海贫血患者、18名重型β地中海贫血患者和17名中间型β地中海贫血症患者。研究组的年龄为3-17岁。20名对照健康受试者被纳入比较,他们的年龄和性别与患者组相匹配。凝胶管用于收集血清进行GDF-15、TGF-β和EPO的酶联免疫吸附试验)。结果:研究组间GDF-15和EPO水平差异极显著(P<0.001)。此外,研究组间TGF-β水平无显著差异(P>0.05)。TGF-β、GDF-15、EPO与严重β地中海贫血患者的脾肿大、肝脾肿大和输血次数无显著相关性(P>05),TGF-β和EPO与中间型β地中海贫血患者的脾肿大、肝脾肿大显著相关,而GDF-15与脾肿大无关。在严重β地中海贫血患者中,EPO与血红蛋白、堆积细胞体积、平均红细胞体积和红细胞计数呈负相关,而GDF-15与淋巴细胞和中性粒细胞计数显著相关。TGF-β与血小板计数显著相关。在中间型β地中海贫血患者中,EPO和GDF-15与任何血液学参数均不相关,而TGF-β与红细胞计数显著相关。结论:与对照组相比,红细胞生成标志物GDF-15、EPO在重型β地中海贫血和中间型β地中海贫血患者中高表达,这可作为抑制无效红细胞生成的未来治疗目标。
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