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目的 观察阵发性睡眠性血红蛋白尿症(PNH)患者骨髓造血细胞对粒细胞集落刺激因子(G CSF)的反应并研究其机制。方法 ①用半固体培养基体外培养17例PNH患者和12名正常人骨髓单个核细胞(BMMNC),观察加与不加G CSF两组粒单核细胞集落(CFU GM)和集簇(cFU GM)形成情况。PNH患者骨髓GPI+CD34+和GPI-CD34+细胞表达粒细胞集落刺激因子受体(G CSFR、CD114)和干细胞生长因子受体(C KIT、CD117)的差异。②用流式细胞术检测20例初发PNH患者和12名正常对照BMMNC和CD34+细胞表面GPI锚定蛋白CD59以及CD114和CD117的表达。结果 ①无G CSF及加G CSF培养PNH组cFU GM数量分别为( 112. 41±22. 74 )和( 133. 82±25. 85 ) /105BMMNC,均较正常对照的(190. 33±36. 05)和(309. 42±92. 94) /105 BMMNC少(P<0. 05);无G CSF及加G CSF培养PNH组CFU GM数量分别为(24. 29±9. 05)和(27. 53±10. 65) /105 BMMNC,也较正常对照的(77. 42±36. 01)和(98. 00±43. 14) /105 BMMNC少(P<0. 05 )。PNH组加G CSF后,cFU GM增加率为(20. 29±6. 82)% (P<0. 05),CFU GM增加率为(16. 45±3. 28)% (P>0. 05)。正常对照加G CSF后,cFU GM增加率为(56. 11±37. 59)%,CFU GM增加率为(48. 03±13. 60)% (P值均<0. 05),PNH组增加率均低于正常对照(P<0
Objective To investigate the response of bone marrow hematopoietic cells to granulocyte colony-stimulating factor (G CSF) in patients with paroxysmal nocturnal hemoglobinuria (PNH) and to investigate its mechanism. Methods ① The bone marrow mononuclear cells (BMMNC) from 17 patients with PNH and 12 normal individuals were cultured in vitro with semi-solid medium. CFU GM and cFU GM The formation of the situation. Difference of granulocyte-colony stimulating factor receptor (G CSFR, CD114) and stem cell growth factor receptor (C KIT, CD117) expression in bone marrow GPI + CD34 + and GPI-CD34 + cells of PNH patients. ② Flow cytometry was used to detect the expression of GPI-anchored protein CD59, CD114 and CD117 on 20 MNH patients and 12 normal control BMMNC and CD34 + cells. Results ① The numbers of cFU GM in PNH group without G CSF plus G CSF were (112. 41 ± 22.74) and (133.8 ± 25.85) / 105 BMMNC, respectively, which were significantly higher than that of normal control (190.33 ± 36 (P <0.05). The numbers of CFU GM in PNH group without G CSF and G CSF supplementation were (24.29 ± 9.55) and (27. 53 ± 10. 65) / 105 BMMNC and also less (77. 42 ± 36. 01) and (98.00 ± 43.14) / 105 BMMNC than the normal controls (P <0.05). The increase rate of cFU GM in PNH group was (20.29 ± 6. 82)% (P <0.05), and the increase rate of CFU GM was (16.45 ± 3.28)% (P> 0. 05). The increase rate of cFU GM was (56.11 ± 37.59)% and the increase rate of CFU GM was (48.03 ± 13.60)% (all P <0.05) in the PNH group Increasing rate was lower than the normal control (P <0