Department of Hematology/Oncology, KonKuk University Hospital, KonKuk University, Seoul, Korea.
Genes Chromosomes Cancer. 2010 Sep;49(9):844-50. doi: 10.1002/gcc.20793.
The characteristics and clinical outcome of 600 adult patients with aplastic anemia (AA) that had successful cytogenetic studies at the time of diagnosis were retrospectively evaluated. Among these, 572 (95.3%) had normal cytogenetics and 28 (4.7%) had abnormal cytogenetics. The most frequent abnormality was trisomy 8 (n = 15), followed by monosomy 7/deletion of 7q (n = 5), and deletion of 1q (n = 5). There were no statistically significant differences with respect to gender, hepatitis viral infection, paroxysmal nocturnal hemoglobinuria, or severity of disease between the patients in the normal and abnormal cytogenetics groups; however, the patients with abnormal cytogenetics were generally younger than those with normal cytogenetics (P < 0.001). Abnormal cytogenetics was associated with a higher cumulative leukemic transformation rate (P < 0.001) and lower leukemic transformation-free survival (P = 0.021). Furthermore, abnormal cytogenetics was an independent predictor of a poor response to immunosuppressive therapy (HR = 0.255; 95% CI = 0.077-0.839; P = 0.024). These analyses suggest that patients with AA and abnormal cytogenetics have different clinical characteristics compared to patients with AA and normal cytogenetics.
回顾性分析了 600 例诊断时成功进行细胞遗传学研究的成人再生障碍性贫血(AA)患者的特征和临床结局。其中,572 例(95.3%)具有正常核型,28 例(4.7%)具有异常核型。最常见的异常是三体 8(n=15),其次是单体 7/7q 缺失(n=5)和 1q 缺失(n=5)。正常核型组和异常核型组患者在性别、肝炎病毒感染、阵发性睡眠性血红蛋白尿或疾病严重程度方面无统计学差异;然而,异常核型患者普遍比正常核型患者年轻(P<0.001)。异常核型与更高的累积白血病转化率(P<0.001)和更低的白血病转化率无事件生存(P=0.021)相关。此外,异常核型是免疫抑制治疗反应不良的独立预测因素(HR=0.255;95%CI=0.077-0.839;P=0.024)。这些分析表明,与正常核型的 AA 患者相比,异常核型的 AA 患者具有不同的临床特征。