Relative increase of granulocytes with a paroxysmal nocturnal haemoglobinuria phenotype in aplastic anaemia patients: the high prevalence at diagnosis
Relative increase of granulocytes with a paroxysmal nocturnal haemoglobinuria phenotype in aplastic anaemia patients: the high prevalence at diagnosis
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DOI：
10.1034/j.1600-0609.2001.00376.x
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发表时间：
2001-03
影响因子：
3.1
通讯作者：
Hongbo Wang;T. Chuhjo;H. Yamazaki;S. Shiobara;M. Teramura;Hideaki Mizoguchi;S. Nakao
中科院分区：
文献类型：
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作者：
Hongbo Wang;T. Chuhjo;H. Yamazaki;S. Shiobara;M. Teramura;Hideaki Mizoguchi;S. Nakao
摘要：为了阐明再生障碍性贫血（AA）患者阵发性睡眠性血红蛋白尿（PNH）表型的粒细胞的病理意义，我们使用灵敏的流式细胞术检测了100例AA患者外周血中糖基磷脂酰肌醇（GPI）锚定蛋白缺陷的粒细胞。与正常个体相比，在35例诊断时未经治疗的AA患者中有31例（88.6%）观察到CD 55 − CD 59 − CD 11b+粒细胞频率显著增加（>0.003%）。病程短（5年）的AA患者中，PNH粒细胞增加的患者比例分别为68.6%（11/16）和20.4%（10/49）。当19例患者治疗前PNH粒细胞频率增加，研究抗胸腺细胞球蛋白加环孢素A治疗后6-12个月，频率下降到0.01-90%的治疗前的值在15个恢复患者。这些研究结果表明，PNH粒细胞数量的相对增加是AA在诊断时的一个共同特征，并且它可能代表了作为AA原因的正常造血干细胞的免疫压力的存在。
Abstract: To clarify the pathologic significance of granulocytes exhibiting the paroxysmal nocturnal haemoglobinuria (PNH) phenotype in patients with aplastic anaemia (AA), we examined peripheral blood from 100 patients with AA for the presence of granulocytes deficient in glycosylphosphatidylinositol (GPI)‐anchored proteins using a sensitive flow cytometric assay. A significant increase in the frequency of CD55−CD59−CD11b+ granulocytes (>0.003%) compared to normal individuals was observed in 31 of 35 (88.6%) patients with untreated AA at diagnosis. The proportions of patients showing increased PNH granulocytes in treated AA patients with a short (5 yr) disease duration were 68.6% (11/16) and 20.4% (10/49), respectively. When 19 patients showing increased frequency of PNH granulocytes before therapy were studied 6–12 months after antithymocyte globulin plus cyclosporin A therapy, the frequency decreased to 0.01–90% of pretreatment values in 15 recovering patients. These findings suggest that a relative increase in the number of PNH granulocytes is a common feature of AA at diagnosis, and that it may represent the presence of immunologic pressure to normal haematopoietic stem cells as a cause of AA.