Minor population of CD55-CD59- blood cells predicts response to immunosuppressive therapy and prognosis in patients with aplastic anemia
Minor population of CD55-CD59- blood cells predicts response to immunosuppressive therapy and prognosis in patients with aplastic anemia
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DOI：
10.1182/blood-2005-06-2485
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发表时间：
2006-02-15
期刊：
影响因子：
20.3
通讯作者：
Nakao, S
中科院分区：
文献类型：
--
作者：
Sugimori, C;Chuhjo, T;Nakao, S
我们研究了获得性再生障碍性贫血(AA)患者中少数阵发性睡眠性血红蛋白尿(PNH)型血细胞的临床意义。我们对122例新近确诊的再生障碍性贫血患者外周血中CD55-CD59-粒细胞和红细胞(RBC)进行了定量，并检测了PNH类型细胞的相关数量和对免疫抑制治疗(IST)的反应。在68%的再生障碍性贫血患者中，流式细胞仪检测到0.005%~23.1%的GPI-AP(-)细胞。83例PNH细胞比例(PNH+)升高的患者中，68例(91%)对抗胸腺细胞球蛋白(ATG)+环孢素(CsA)治疗有反应，而39例无此升高(PNH-)的患者中有18例(48%)有效。5年后，PNH+患者的无失败存活率(%)显著高于PNH-患者(12%)，尽管两组总体存活率相似。在大多数对IST有反应的PNH+患者中，PNH类型细胞和正常类型细胞的数量平行增加，这表明这些细胞对免疫攻击同样敏感。这些结果表明，少量PNH型细胞是再生障碍性贫血患者IST反应阳性的可靠标志和良好的预后。此外，对允许PNH克隆性扩增的造血干细胞的免疫攻击可能仅在再生障碍性贫血发病时发生。
We investigated the clinical significance of a minor population of paroxysmal nocturnal hemoglobinuria (PNH)-type blood cells in patients with acquired aplastic anemia (AA). We quantified CD55-CD59-granulocytes and red blood cells (RBCs) in peripheral blood from 122 patients with recently diagnosed AA and correlated numbers of PNH-type cells and responses to immunosuppressive therapy (IST). Flow cytometry detected 0.005% to 23.1% of GPI-AP(-) cells in 68% of patients with AA. Sixty-eight of 83 (91%) patients with an increased proportion of PNH-type cells (PNH+) responded to antithymocyte globulin (ATG) + cyclosporin (CsA) therapy, whereas 18 of 39 (48%) without such an increase (PNH-) responded. Failure-free survival rates were significantly higher (64%) among patients with PNH+ than patients with PNH- (12%) at 5 years, although overall survival rates were comparable between the groups. Numbers of PNH-type and normal-type cells increased in parallel among most patients with PNH+ who responded to IST, suggesting that these cells are equally sensitive to immune attack. These results indicate that a minor population of PNH-type cells represents a reliable marker of a positive IST response and a favorable prognosis among patients with AA. Furthermore, immune attack against hematopoietic stem cells that allows PNH clonal expansion might occur only at the onset of AA.