阵发性睡眠性血红蛋白尿症患者骨髓正常细胞及异常克隆分析

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 （1）PNH患者骨髓正常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CD59^ 细胞总数较异常者显著减少，淋巴细胞窗及粒细胞窗内CD59^ 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CD59^ 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。

Analysis of Cell Components of CD59+ Normal Cells and Abnormal Clons in Bone Marrow of Patients with Paroxysmal Nocturnal Hemoglobinuria

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: 1. There was significant difference in BMNCs between normal CD59+ cells and abnormal clons (CD59-) cells, the normal CD59+ BMNCs were mainly composed of the cells in lymphocyte-window, while the abnormal CD59- BMNCs were mainly in blast window and in granulocyte-window. 2. The CD34+ cells in CD59+ BMNCs were also markedly decreased when compared with their CD59- counterparts, and the decrease of CD34+ cells in CD59+ BMNCs was more obvious in lymphocyte window than that in blast window. CONCLUSION: The constituents of BMNCs and the amount of CD34+ cells in CD59+ and CD59- 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.