阵发性睡眠性血红蛋白尿症患者基质研究初探  

Study on the stroma of patients with paroxysmal nocturnal hematoglobinuria

作　　者：韩冰[1] 武永吉[1] 张之南[1] 

机构地区：[1]中国医学科学院中国协和医科大学北京协和医院血液科,100730

出　　处：《中华医学杂志》2001年第23期1430-1433,共4页National Medical Journal of China

基　　金：卫生部重点基金资助项目 ( 970 10 2 0 1)

摘　　要：目的　研究与造血密切相关的基质在阵发性睡眠性血红蛋白尿症 (PNH)患者中是否存在异常。方法　对 7例患者骨髓进行成纤维细胞集落 (CFU F)的培养 ,观察其形态及数量是否存在异常 ;将分选后的CD34 + 正常造血干 /祖细胞分别加入含有相同数量的患者及正常对照基质的培养基中 ,观察其扩增及形成集落的情况 ;用半定量RT PCR的方法 ,以自身 β 肌动蛋白作为内参对照 ,检测患者肿瘤坏死因子 (TNF) α、白细胞介素 6 (IL 6 )表达量与正常人是否不同。结果　 (1)PNH患者骨髓CFU F数量 (13 0个± 1 8个 )类似正常人 (10 7个± 3 7个 ,P >0 0 5 )。 (2 )PNH患者的骨髓基质细胞对正常人造血干 /祖细胞支持能力与正常人相仿。液体培养细胞数为 :(4 2 1± 1 0 5 )个× 10 4 vs(4 76± 1 0 4)个× 10 4 ;半固体培养粒细胞集落 :10 80个± 5 40个vs 1 5 0个± 0 2 3个 ;红细胞集落形成单位 :4 5 0个± 3 5 1个vs 0 83个± 0 12个 ,P >0 0 5 )。 (3)PNH患者骨髓基质细胞的TNF α(以细胞因子与自身β 肌动蛋白电泳条带强度的比值表示 :0 5 5± 0 12 )和IL 6 (0 6 2± 0 13)mRNA表达量与正常人 (0 48± 0 0 8、0 6 8± 0 10 )相似 (P >0 0 5 )。结论　患者的骨髓成纤维细胞集落形成能力是正常的。患?Objective To investigate the relationship between the stroma of bome marrow and the abnormality of hematopoiesis in patients with paroxysmal nocturnal hematoglobinuria (PNH) Methods The bone marrow stromata from PNH patients was compared with those from normal volunteers in aspects of their CFU F formation and hematopoietic sustentation for normal CD34 + cells The stromal IL 6 and TNF α mRNA expression in patients and normal volunteers was also measured by RT PCR(self β actin as contrast) Results (1) The CFU F formation ability in PNH patients was very similar to that in normal volunteers ( P >0 05) (2) The bone marrow stromata from patients with PNH had the same hematopoietic maintainence compared to those from normal controls ( P >0 05) (3)The stomal IL 6 and TNF α mRNA expression was similar in PNH patients and in normal volunteers ( P >0 05) Conclusion The CFU F formation capacity of bone marrow fibroblast was normal in PNH patients The stromata in patients with PNH had normal hematopoietic maintainence to the normal CD34 + cells The mRNA expression of IL 6 and TNF α in PNH stromata was almost normal

关 键 词：阵发性血红蛋白尿症 造血系统 PNH 成纤维细胞 骨髓CD34^+细胞 

分 类 号：R556.7[医药卫生—血液循环系统疾病]