Butthep Punnee, Wisedpanichkij Raewadee, Jindadamrongwech Sumalee, Fucharoen Suthat
Hematology Laboratory, Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Hematology Laboratory, Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Blood Cells Mol Dis. 2015 Feb;54(2):170-6. doi: 10.1016/j.bcmd.2014.11.007. Epub 2014 Nov 26.
Serum EPO concentration is related primarily to the rate of erythrocyte production and, under the stimulation of hypoxia, increases exponentially as hemoglobin (Hb) decreased. The level of EPO was determined in 141 subjects including 43 normal, 44 thalassemic patients and 54 thalassemic trait subjects. The EPO level was significantly higher in the thalassemic patients (54.8mU/ml in HbH disease [α thal1/α thal2;], 78.1mU/ml in HbH with Hb CS [α thal 1/CS]; 95.6mU/ml in β-thal/HbE splenectomized [BE(S)]; and 114.8mU/ml in β-thal/HbE non-splenectomized [BE(NS)]as compared with 12.0mU/ml in normal subjects. No significant differences were detected in thalassemic trait subjects. In addition, the levels of EPO in thalassemic patients is correlated significantly with the number of reticulocytes and the reticulocyte fractions especially the fraction of immature reticulocytes. Interestingly, the highest level of EPO/% retic ratio as indicated for EPO non-responder was detected in BE(NS) patients. However, the impaired reticulocytes maturation was found to be related significantly with the levels of TNF-α,IFN-γ,IL-10, and VEGF. Since, TNF-α, IFN-γ, IL-10 and VEGF are reported as the cytokines with erythropoietic inhibitory mediators, the variation of these cytokines in thalassemic environments may be associated to the anemic crisis in these patients.
血清促红细胞生成素（EPO）浓度主要与红细胞生成速率相关，在缺氧刺激下，随着血红蛋白（Hb）水平降低呈指数增加。在141名受试者中测定了EPO水平，其中包括43名正常人、44名地中海贫血患者和54名地中海贫血特征携带者。地中海贫血患者的EPO水平显著高于正常人（正常受试者为12.0mU/ml，血红蛋白H病[α地贫1/α地贫2]患者为54.8mU/ml；合并血红蛋白CS的血红蛋白H病[α地贫1/CS]患者为78.1mU/ml；β地中海贫血/HbE脾切除患者[BE(S)]为95.6mU/ml；β地中海贫血/HbE未脾切除患者[BE(NS)]为114.8mU/ml）。在地中海贫血特征携带者中未检测到显著差异。此外，地中海贫血患者的EPO水平与网织红细胞数量和网织红细胞比例显著相关，尤其是未成熟网织红细胞比例。有趣的是，在BE(NS)患者中检测到EPO无反应者的EPO/网织红细胞比例最高。然而，发现网织红细胞成熟受损与肿瘤坏死因子-α（TNF-α）、干扰素-γ（IFN-γ）、白细胞介素-10（IL-10）和血管内皮生长因子（VEGF）水平显著相关。由于TNF-α、IFN-γ、IL-10和VEGF被报道为具有促红细胞生成抑制介质作用的细胞因子，这些细胞因子在地中海贫血环境中的变化可能与这些患者的贫血危机有关。
