Therapy-related leukemia and myelodysplastic syndrome: a large-scale Japanese study of clinical and cytogenetic features as well as prognostic factors.
Therapy-related leukemia and myelodysplastic syndrome: a large-scale Japanese study of clinical and cytogenetic features as well as prognostic factors.
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治疗相关白血病和骨髓增生异常综合征：日本一项针对临床和细胞遗传学特征以及预后因素的大规模研究。
DOI：
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发表时间：
2000
影响因子：
2.1
通讯作者：
R. Ueda
中科院分区：
文献类型：
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作者：
K. Takeyama;M. Seto;N. Uike;N. Hamajima;T. Ino;C. Mikuni;Toshitaka Kobayashi;A. Maruta;Yoshitomi Muto;Noboru Maseki;H. Sakamaki;Hidehiko Saitoh;M. Shimoyama;R. Ueda
已知烷化剂和拓扑异构酶II抑制剂可引起不同形式的治疗相关白血病和骨髓增生异常综合征（TRL/MDS）。虽然已经分别对每种药物进行了几次报告，但尚未进行研究来评估这两种药物在同一人群中的作用。在一项全国性的大规模人群研究中，对256例TRL/MDS患者的临床和细胞遗传学特征以及预后因素进行了评估。中位年龄为61岁，原发性恶性肿瘤的中位潜伏期为47.9个月。在接受化疗的患者中，潜伏期明显缩短，特别是拓扑异构酶II抑制剂，为原发性癌症。TRL/MDS的形态学诊断为急性髓系白血病占59%，MDS占41%。在189名接受检查的患者中，77%的患者记录了经常涉及5号、7号或11号染色体的染色体异常。在58例受试者中有11例检测到MLL基因重排，并且与拓扑异构酶II抑制剂给药的临界显著性（P = 0.072）相关。总体中位生存期仅为9.7个月。有或无MLL基因重排的病例生存率相似。多因素分析显示5号染色体异常、低蛋白血症、原发癌治疗效果差、C反应蛋白和血小板减少是显著的不良预后因素（P < 0.05）。这项大规模人群研究提供了日本TRL/MDS状态的全面更新，确定了重要的预后因素，并能够评估MLL基因重排的临床意义。
It is known that alkylating agents and topoisomerase II inhibitors can cause distinct forms of therapy-related leukemia and myelodysplastic syndrome (TRL/MDS). Although several reports have been made on each of these agents separately, no study has yet been conducted to evaluate the effect of these two types of agents in the same population. In a nationwide, large-scale population study, the clinical and cytogenetic features as well as the prognostic factors in 256 patients with TRL/MDS were assessed. Median age was 61 years, and the median period of latency from primary malignancies was 47.9 months. The latency period was significantly shorter in patients undergoing chemotherapy, especially that of topoisomerase II inhibitors, for primary cancer. The morphological diagnosis of TRL/MDS was acute myeloid leukemia in 59% and MDS in 41% of patients. Chromosome abnormalities that frequently involved chromosomes 5, 7 or 11 were documented in 77% of the 189 patients examined. MLL gene rearrangements were detected in 11 of 58 subjects and were correlated with a borderline significance (P = 0.072) with topoisomerase II inhibitor administration. Overall median survival was only 9.7 months. Survival was similar in cases with or without MLL gene rearrangement. Multivariate analysis identified chromosome 5 abnormalities, hypoproteinemia, poor therapy outcomes for primary cancer, C-reactive protein, and thrombocytopenia as being significantly poor prognostic factors (P < 0.05). This large-population study provided a comprehensive update of TRL/MDS status in Japan, identified significant prognostic factors, and enabled the clinical significance of MLL gene rearrangement to be assessed.