PNH患者外周血及骨髓正常和异常造血干/祖细胞组成分析

目的　研究阵发性睡眠性血红蛋白尿症 (PNH)患者外周血及骨髓造血干 祖细胞的组成及含量。方法　用流式细胞仪测定 2 1例PNH患者外周血及骨髓造血干 祖细胞 (以CD3 4+为标志 )锚连蛋白CD59的表达 ,并将患者造血干 祖细胞总数与正常人进行比较。结果　PNH患者骨髓及外周血CD3 4+细胞总量均明显低于正常人 (P <0 .0 0 1) ,但在疾病缓解时总量与正常对照差异无显著性 (P >0 0 5 )。PNH患者外周血CD3 4+细胞主要以CD59+表型为主 CD3 4+CD59+细胞占总CD3 4+细胞的 (81.4±16 1) % ],临床未缓解或骨髓中CD3 4+细胞以CD59- 表达为主的患者 ,其外周血CD3 4+细胞仍以CD59+表型为主。结论　PNH患者造血干 祖细胞含量明显减少 ,但在外周血中以正常表型为主

Composition analysis of hematopoietic stem/progenitor cells in peripheral blood and bone marrow from pafients with paroxymal nocturnal hemoglobinuria

Objective To evaluate the composition and proportion of the hematopoietic stem cells in peripheral blood (PB) and bone marrow (BM) in paroxysmal nocturnal hemoglobinuria (PNH) patients. Methods The CD 34 and CD 59 expressions in PB and BM samples from 21 PNH patients and 8 normal volunteers were analyzed by flow cytometry, and their total numbers were compared. Results The number of CD 34 + cells in both PB and BM of the patients was significantly lower than that in normal controls (P<0.001), while there was no significant difference between the remittent patients and normal controls. The CD 34 + cells in PB of the patients exhibited predominantly normal CD 59 phenotype, which had no relationship either to the patients' clinical manifestations or to the CD 59 expression status of bone marrow CD 34 + cells. Conclusion The hematopoietic stem cells in patients with PNH decreases in number but show mainly normal phenotype in PB.