Persistence of affected T lymphocytes in long-term clinical remission in paroxysmal nocturnal hemoglobinuria.
Persistence of affected T lymphocytes in long-term clinical remission in paroxysmal nocturnal hemoglobinuria.
复制标题
阵发性睡眠性血红蛋白尿症的长期临床缓解中受影响的 T 淋巴细胞的持续存在。
作者：
H. Nakakuma;S. Nagakura;T. Kawaguchi;N. Iwamoto;M. Hidaka;K. Horikawa;T. Kagimoto;Ryuichiro Tsuruzaki;K. Takatsuki
阵发性睡眠性血红蛋白尿症（PNH）临床缓解时间超过10年的病例很少。PNH患者的血细胞缺乏糖基磷脂酰肌醇（GPI）锚定的膜蛋白，如衰变加速因子（decay-accelerating factor，CD 59）。我们进行了流式细胞仪分析循环血细胞从两个PNH患者谁已经在临床缓解超过10年和25年，分别获得。PNH表型的受影响细胞仅在T淋巴细胞中表现出来。持续受影响的T细胞仅对CD 52蛋白呈阴性，该蛋白是没有补体调节活性的GPI锚定的淋巴细胞标志物。受影响的T细胞的持久性可以通过PNH干细胞消失后固有的长寿命或通过受影响的干细胞在亚临床水平的潜伏性生产来解释。在这两种情况下，检测受影响的T细胞，特别是CD 52阴性T细胞，可能是有用的长期临床缓解的评估PNH。
Long-term clinical remission of more than 10 years is rarely seen in paroxysmal nocturnal hemoglobinuria (PNH). Affected blood cells in PNH lack glycosylphosphatidylinositol (GPI)-anchored membrane proteins such as decay-accelerating factor (DAF) and CD59. We performed a flow cytometric analysis of circulating blood cells obtained from two patients with PNH who had been in clinical remission for more than 10 and 25 years, respectively. Affected cells with the PNH phenotype were demonstrated only among T-lymphocytes. Persistent affected T cells were negative for the CD52 protein only, this protein being a GPI-anchored lymphocyte marker without complement regulatory activity. The persistence of the affected T cells may be explained either by an inherently long life span after the disappearance of the PNH stem cell or by insidious production at a subclinical level by affected stem cell. In either event, detection of affected T cells, especially CD52-negative T cells, may be useful for the evaluation of long-term clinical remission in PNH.
阵发性睡眠性血红蛋白尿粒细胞中糖基磷脂酰肌醇锚合成缺陷。
影响因子：
20.3
作者：
Mahoney,JF;Urakaze,M;Hall,S;DeGasperi,R;Chang,HM;Sugiyama,E;Warren,CD;Borowitz,M;Nicholson-Weller,A;Rosse,WF
通讯作者：
Rosse,WF