阵发性睡眠性血红蛋白尿症患者CD59＋与CD59-骨髓造血干细胞EPOR、TPOR表达研究

EPOR and TPOR expressions on CD34 ＋ CD59 - and CD34 ＋ CD59 ＋ bone marrow cells from patients with paroxysmal nocturnal hemoglobinuria

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＋CD59-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-CD59-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、TPORmRNA的表达。结果@PNH患者骨髓CD34＋CD59＋细胞EPOR表达率[（30．67±18．30）％]明显高于CD34＋CD59-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＋CD59-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＋CD59-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＋CD59-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）。（3）PNH患者骨髓单个核细胞EPORmRNA表达水平（0．41±0．37）、TPORmRNA表达水平（0．32±0．19）与对照组骨髓单个核细胞EPOR、TPORmRNA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＋CD59-细胞。

Objective To study the expressions of erythropoietin receptor （EPOR） and thrombopoietin receptor （TPOR） on CD34 ＋ CD59- and CD34 ＋ CD59 ＋ bone marrow （BM） cells from patients with paroxysmal nocturnal hemoglobinuria（PNH）. Methods （1）The expressions of EPOR and TPOR on CD34 ＋CD59 ＋ and CD34 ＋ CD59 - BM cells from 26 PNH patients and 16 normal controls were examined by flow cytometry （FCM）. （2）The 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 （1）The percentage of EPOR positive cells in PNH CD34 ＋ CD59 ＋ BMMNC [ （30.67 ± 18.30） % ] was significantly higher than that inPNH CD34 ＋CD59 BMMNC [（8.05±3.51）%J （P〈0.01） and than that in control CD34 ＋CD59 ＋ BMMNC [ （8.24 ± 6.51 ） % J （P 〈 0.01 ）, but there was no obvious difference between the CD34 ＋ CD59 - 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 ＋ CD59 BMMNC [（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＋ CD59- BMMNC in PNH and CD34 ＋ CD59 ＋ BMMNC in control. （3）There 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 ＋ CD59- cells. The difference may be due to abnormal transcription of both receptor coding genes.