Chansai Siriyakorn, Yamsri Supawadee, Fucharoen Supan, Fucharoen Goonnapa, Teawtrakul Nattiya
Medical Science Program, Faculty of Associated Medical Sciences, Khon Kaen University Khon Kaen 40002, Thailand.
Centre for Research and Development of Medical Diagnostic Laboratories, Faculty of Associated Medical Sciences, Khon Kaen University Khon Kaen 40002, Thailand.
Am J Transl Res. 2022 Jul 15;14(7):4743-4756. eCollection 2022.
The degree of ineffective erythropoiesis is known to be associated with clinical severity among individuals with thalassemia. The association of ineffective erythropoiesis biomarker levels with different thalassemia genotypes, however, remains limited. The aim of this study was to explore the level of phosphatidylserine-exposed red blood cells (PS-exposed RBCs) and ineffective erythropoiesis biomarkers (growth-differentiation factor-15 and soluble transferrin receptors) in patients with different genotypes.
A cross-sectional study was conducted on 139 patients of age 18 years and above with different genotypes at Srinagarind Hospital, Khon Kaen University, Thailand. The levels of PS-exposed RBCs were determined using flow cytometry. Measurements of growth-differentiation factor-15 (GDF-15) and soluble transferrin receptors (sTfR) were evaluated by the ELISA method.
The PS-exposed RBCs levels were found to be significantly higher in splenectomized beta-thalassemia patients. Patients with beta-thalassemia had the highest GDF-15 levels, followed by patients with non-deletional alpha-thalassemia. Patients with non-deletional alpha-thalassemia showed elevated hemoglobin levels and reduced GDF-15 levels after splenectomy. Patients with beta-thalassemia and non-deletional alpha-thalassemia had the highest levels of PS-exposed RBCs and ineffective erythropoiesis biomarkers, which correlated with the clinical severity of thalassemia.
The levels of ineffective erythropoiesis biomarkers were different across thalassemia genotypes. Splenectomy may improve clinical symptoms of patients with non-deletional alpha thalassemia but not of patients with beta-thalassemia. These findings demonstrate differences in the degree of ineffective erythropoiesis in thalassemia, which emphasizes the need for different treatment approaches among patients with different thalassemia genotypes.
已知无效红细胞生成程度与地中海贫血患者的临床严重程度相关。然而，无效红细胞生成生物标志物水平与不同地中海贫血基因型之间的关联仍然有限。本研究的目的是探讨不同基因型患者中磷脂酰丝氨酸暴露红细胞（PS暴露红细胞）水平以及无效红细胞生成生物标志物（生长分化因子-15和可溶性转铁蛋白受体）水平。
在泰国孔敬大学诗里拉吉医院对139例18岁及以上不同基因型的患者进行了一项横断面研究。使用流式细胞术测定PS暴露红细胞水平。通过ELISA法评估生长分化因子-15（GDF-15）和可溶性转铁蛋白受体（sTfR）。
发现脾切除的β地中海贫血患者的PS暴露红细胞水平显著更高。β地中海贫血患者的GDF-15水平最高，其次是非缺失型α地中海贫血患者。非缺失型α地中海贫血患者脾切除后血红蛋白水平升高，GDF-15水平降低。β地中海贫血和非缺失型α地中海贫血患者的PS暴露红细胞水平和无效红细胞生成生物标志物水平最高，这与地中海贫血的临床严重程度相关。
不同地中海贫血基因型的无效红细胞生成生物标志物水平不同。脾切除可能改善非缺失型α地中海贫血患者的临床症状，但不能改善β地中海贫血患者的症状。这些发现表明地中海贫血中无效红细胞生成程度存在差异，这强调了不同地中海贫血基因型患者需要不同治疗方法。
