【摘要】 目的　分析阵发性睡眠性血红蛋白尿症 (PNH)患者骨髓正常细胞 (CD59+ )及异常克隆细胞 (CD59- )的细胞构成。方法　骨髓有核细胞免疫荧光标记后用流式细胞术分析。结果　 1PNH患者骨髓正常细胞及异常克隆的细胞组成在有核细胞水平是显著不均衡的 ,正常的 CD59+ 细胞以淋巴细胞窗内细胞为主 ,异常的 CD59- 细胞则以粒细胞窗及原始细胞窗内细胞为主。 2 PNH患者骨髓 CD59+正常细胞群所含 CD34 +细胞总数较异常者显著减少 ,淋巴细胞窗及粒细胞窗内 CD34 + 细胞均较异常者显著减少 ,前者减少更为显著。结论　 PNH患者骨髓正常细胞及异常克隆的细胞构成在有核细胞水平及 CD34 + 细胞水平都有显著差异 ,正常造血呈衰竭状态。因此 ,在考虑用患者自体骨髓体外净化去除异常克隆后回输给患者重建造血时 ,应对正常造血细胞作进一步处理 ,否则正常造血细胞可能不具备重建造血的能力更多还原

【Abstract】 Objective To explore the cell components of normal cells and abnormal clons in bone marrow of patients with paroxysmal nocturnal hemoglobinuria (PNH). Methods Bone marrow nuclear cells ( BMNCs ) were labeled by immunofluorescence and analyzed by flow cytometry. Results ①There was significant difference in BMNCs between normal CD 59 + cells and abnormal clons (CD 59 -)cells, the normal CD 59 + BMNCs were mainly composed of the cells in lymphocyte window, while the abnormal CD 59 - BMNCs were mainly in blast window and in granulocyte window. ②The CD 34 + cells in CD 59 + BMNCs were also markedly decreased when compared with their CD 59 - counterparts, and the decrease of CD 34 + cells in CD 59 + BMNCs was more obvious in lymphocyte window than that in blast window. Conclusion The constituents of BMNCs and the amount of CD 34 +cells in CD 59 + and CD 59 - cell populations of PNH patients are significantly different, the normal hematopoiesis is exhausted. On account of the defects in normal hematopoietic cells in patients with PNH, the feasibility of using autologous bone marrow transplantation following purging of abnormal clons as a treatment of PNH should be carefully judged, the normal hematopoietic cells alone might not be able to sustain adequate hematopoiesis without further manipulations.更多还原