Objective: To confirm the presence of erythropoietin receptor (EPOR) antibody in patients with immune-related pancytopenia (IRP) and to evaluate the significance of EPOR in IRP. Methods: A total of 59 newly-diagnosed IRP patients, 62 patients with IRP in remission, 14 patients with aplastic anemia (AA), 15 patients with myelodysplastic syndromes (MDS) and 33 healthy controls were enrolled in this study from January 2013 to June 2015 in Tianjin Medical University General Hospital. The anti-EPOR antibody was detected by enzyme-linked immunosorbent assay(ELISA). The expression of EPOR mRNA was detected by quantitative real-time PCR (qRT-PCR). The correlation between the data and the clinical indicators of patients was analyzed. Results: The levels of anti-EPOR antibodies were higher in the newly-diagnosed IRP patients than in the remission IRP group, AA group, MDS group and controls(0.84±0.39 vs 0.46±0.25, 0.49±0.25, 0.50±0.25, 0.53±0.14, all P<0.05). The expression of EPOR- mRNA was up-regulated in the newly-diagnosed IRP group than in the remission IRP group and the controls. The positive rate of EPOR antibody was significantly elevated in the patients with low hemoglobin level (<100 g/L), positive GlycoA antibody, or low complement C3 (all P<0.05). Anti-EPOR antibody was significantly decreased in the IRP patients responding to immunosuppressive therapy. Conclusions: Anti-EPOR autoantibody is present in patients with IRP. Detection of anti-EPOR antibody has important clinical value in the diagnosis, differential diagnosis and treatment effect evaluation of IRP.
目的: 证实免疫相关性全血细胞减少症(IRP)患者存在促红细胞生成素受体(EPOR)抗体,评价检测EPOR抗体在IRP中的意义。 方法: 选取天津医科大学总医院2013年1月至2015年6月就诊的59例初治IRP患者,62例恢复期IRP患者,14例再生障碍性贫血(AA)患者,15例骨髓增生异常综合征(MDS)患者和33名健康对照,应用酶联免疫吸附试验(ELISA)法检测患者血清中EPOR抗体,实时定量PCR(qRT-PCR)法检测EPOR的mRNA表达水平,并与患者临床指标进行相关性分析。 结果: 初治IRP患者EPOR抗体高于恢复期IRP患者、AA患者、MDS患者及健康对照(EPOR结合指数:0.84±0.39比0.46±0.25、0.49±0.25、0.50±0.25、0.53±0.14,均P<0.05),且EPOR-mRNA表达上调。低血红蛋白、有核红细胞(GlycoA)抗体阳性和低补体C3的IRP患者EPOR抗体阳性率显著高(均P<0.05)。随免疫抑制治疗有效,血细胞恢复,IRP患者EPOR抗体明显下降。 结论: IRP患者存在EPOR自身抗体,检测EPOR抗体对IRP的诊断、鉴别及评价治疗效果有重要临床价值。.
Keywords: Anemia; Autoantibody; Bone marrow failure; Immuno-related pancytopenia; Receptors, erythropoietin.