Nakakuma H, Nagakura S, Kawaguchi T, Iwamoto N, Hidaka M, Horikawa K, Kagimoto T, Tsuruzaki R, Takatsuki K
Second Department of Internal Medicine, Kumamoto University School of Medicine, Japan.
Blood. 1994 Dec 1;84(11):3925-8.
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（PNH）很少出现超过10年的长期临床缓解情况。PNH中受影响的血细胞缺乏糖基磷脂酰肌醇（GPI）锚定膜蛋白，如衰变加速因子（DAF）和CD59。我们对分别处于临床缓解超过10年和25年的两名PNH患者的循环血细胞进行了流式细胞术分析。具有PNH表型的受影响细胞仅在T淋巴细胞中被证实。持续存在的受影响T细胞仅对CD52蛋白呈阴性，该蛋白是一种无补体调节活性的GPI锚定淋巴细胞标志物。受影响T细胞的持续存在可能是由于PNH干细胞消失后其固有寿命较长，或者是由于受影响干细胞在亚临床水平隐匿产生。无论哪种情况，检测受影响的T细胞，尤其是CD52阴性T细胞，可能有助于评估PNH的长期临床缓解情况。
