阵发性睡眠性血红蛋白尿症患者骨髓造血细胞对粒细胞集落刺激因子反应的研究  

The response of bone marrow hematopoietic cells to G-CSF in paroxysmal nocturnal hemoglobinuria patients

作　　者：曹燕然[1] 邵宗鸿[1] 刘鸿[1] 施均[1] 白洁[1] 涂梅峰[1] 王化泉[1] 邢莉民[1] 崔振珠[1] 孙娟[1] 贾海蓉[1] 杨天楹[1] 

机构地区：[1]中国医学科学院,中国协和医科大学血液学研究所,血液病医院,天津300020

出　　处：《中华血液学杂志》2005年第4期235-238,共4页Chinese Journal of Hematology

摘　　要：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)。Objective To study the response of hematopoietic cells (HSC) to granulocyte colony stimulating factor (G-CSF) in paroxysmal nocturnal hemoglobinuria (PNH) patients. Methods ①Bone marrow mononuclear cells (BMMNC) from 17 PNH patients and 12 normal subjects were inoculated into semisolid culture media containing or not G-CSF (50 ng/ml). The cluster/colony forming unit-granulocyte/monocyte (CFU/cFU-GM) were counted and compared. ②BMMNC of 20 PNH patients and 12 normal controls were triply stained for CD34, CD59 and G-CSF receptor CD114/stem cell factor receptor (C-KIT) CD117 and assessed by FCM. The CD34 + cells were identified as CD34 + /CD59 + and CD34 +/CD59 -. Percentage of CD114 and CD117 expression in each cell population was calculated. Results ①PNH cFU- GM without G-CSF were (112.41±22.74)/10 5 BMMNC, while with G-CSF:(133.82±25.85)/10 5 BMMNC and normal cFU-GM were ( 190.33 ±36.05)/10 5 BMMNC, (309.42±92.94)/10 5 BMMNC, respectively. Whether with or without G-CSF, PNH BMMNC formed less cFU-GM than control did, both of the two kinds of BMMNC responded to G-CSF well(P<0.05), but the increment of PNH cFU-GM yields was less than that of the normal control(P<0.05). CFU-GM yields of PNH BMMNC without G-CSF were ( 24.29± 9.05)/10 5 BMMNC,with G-CSF were (27.53±10.65)/10 5 BMMNC, while normal control were ( 77.42± 36.01)/10 5 BMMNC and (98.00±43.14)/10 5 BMMNC, respectively. Whether with or without G-CSF, PNH BMMNC showed less CFU-GM yields than that of control(P<0.05). ②The percentage of CD114 positive cells in PNH CD34 + CD59 + BMMNC was (73.34±29.40)% and that in PNH CD34 + CD59 - BMMNC and in control CD34 +CD59 + BMMNC were (32.70±6.89)% and (58.52±29.99)%, respectively. The percentage of CD114 expression in PNH CD34 + CD59 - BMMNC was less than that in the other two groups (P<0.05). The percentages of CD117 positivities on the PNH CD34 +CD59 + BMMNC were (76.90± 22.08 )%, PNH CD34 + CD59 - (36.03±7.69)% and control C

关 键 词：PNH G-CSF 患者 CFU-GM 正常 CD34^+CD59^+细胞 对照 表达率 骨髓造血细胞 克隆 

分 类 号：R55[医药卫生—血液循环系统疾病]