摘要目的 研究阵发性睡眠性血红蛋白尿症(PNH)患者骨髓CD34+CD59+和CD34+CD59ˉ细胞红细胞生成素受体(EPOR)和血小板生成素受体(TPOR)的表达.方法 ①应用流式细胞术检测26例PNH患者和16名正常对照者骨髓CD34+CD59+和CD34+CD59ˉ细胞EPOR、TPOR的表达情况.②应用RT-PCR方法检测25例PNH患者和13名正常对照者骨髓单个核细胞EPOR、TPOR mRNA的表达.结果 ①PNH患者骨髓CD34+CD59+细胞EPOR表达率[(30.67±18.30)%]明显高于CD34+CD59ˉ细胞[(8.05±3.51)%](P＜0.01)及对照组CD34+CD59+细胞[(8.24±6.51)%](P＜0.01),但PNH患者骨髓CD34+CD59ˉ细胞与对照组CD34+CD59+细胞EPOR表达率差异无统计学意义(P＞0.05).②PNH患者骨髓CD34+CD59+细胞TPOR表达率[(28.15±17.75)%]明显高于CD34+CD59ˉ细胞[(15.65±14.45)%](P＜0.05)及对照组CD34+CD59+细胞[(10.77±8.39)%](P＜0.01),但PNH患者骨髓CD34+CD59ˉ细胞与对照组CD34+CD59+细胞TPOR表达率差异无统计学意义(P＞0.05).③PNH患者骨髓单个核细胞EPOR mRNA表达水平(0.41±0.37)、TPOR mRNA表达水平(0.32±0.19)与对照组骨髓单个核细胞EPOR、TPOR mRNA表达水平[(0.47±0.33)、(0.40±0.29)]比较,差异均无统计学意义.结论 PNH患者骨髓CD34+CD59+细胞EPOR、TPOR表达明显高于CD34+CD59ˉ细胞,该差别可能源于受体基因转录后表达异常.

abstractsObjective To study the expressions of erythropoietin receptor (EPOR) and thrombopoietin receptor (TPOR) on CD34 + CD59ˉ and CD34 + CD59 + bone marrow (BM) cells from patients with paroxysmal nocturnal hemoglobinuria(PNH).Methods ①The expressions of EPOR and TPOR on CD34+CD59 + and CD34 + CD59 ˉ BM cells from 26 PNH patients and 16 normal controls were examined by flow cytometry (FCM).②The mRNA expression of the EPOR and the TPOR in BM mononuclear cells(BMMNC) from 25 PNH patients and 13 normal controls were examined by RT-PCR.Results ①The percentage of EPOR positive cells in PNH CD34 + CD59 + BMMNC[(30.67 ± 18.30)%]was significantly higher than that in PNH CD34+CD59ˉ BMMNC[(8.05 ±3.51 )%]( P ＜0.01 ) and than that in control CD34+ CD59+BMMNC[(8.24 ±6.51 )%](P＜0.01 ), but there was no obvious difference between the CD34+ CD59ˉ BMMNC in PNH and CD34+ CD59+ BMMNC in control.② The percentage of TPOR positive cells in PNH CD34+ CD59+ BMMNC[(28.15 ± 17.75)%]was significantly higher than that in PNH CD34+ CD59ˉBMMNC[( 15.65 ± 14.45) %]( P ＜ 0.05 ) and than that in control CD34 + CD59+ BMMNC[( 10.77 ±8.39) %](P ＜ 0.01 ), but there was no obvious difference between the CD34 + CD59ˉ BMMNC in PNH and CD34 + CD59 + BMMNC in control.③There was no statistic difference in EPOR mRNA and TPOR mRNA expressions in BMMNCs between PNH patients group[(0.41 ±0.37) and (0.32 ±0.19), respectively]and control group[(0.47 ±0.33) and (0.40 ±0.29), respectively].Conclusion The expression of EPOR and TPOR of PNH patients on BM CD34 + CD59 + cells are significantly higher than those on BM CD34 + CD59ˉ cells.The difference may be due to abnormal transcription of both receptor coding genes.