Forestier E, Johansson B, Gustafsson G, Borgström G, Kerndrup G, Johannsson J, Heim S
Department of Paediatrics, University of Umeå, Sweden.
Br J Haematol. 2000 Jul;110(1):147-53. doi: 10.1046/j.1365-2141.2000.02153.x.
The prognostic impact of acquired chromosome abnormalities was evaluated in a population-based consecutive series of 768 children (< 15 years of age) with acute lymphoblastic leukaemia (ALL). The study cohort included all cases of cytogenetically abnormal childhood ALL diagnosed between 1986 and 1997 in the five Nordic countries (Denmark, Finland, Iceland, Norway and Sweden). The probability of event-free survival (pEFS) for the total cohort was 0. 72 +/- 0.02. When comparing the two treatment periods of July 1986 to December 1991 and January 1992 to December 1997, a better survival was seen for the latter time period (pEFS of 0.69 +/- 0.02 vs. 0.76 +/- 0.02, P = 0.05). Hypodiploidy with less than 45 chromosomes, t(9;22)(q34;q11) and 11q23 translocations were associated with a dismal outcome during the whole study period (pEFS of 0.57 +/- 0.12, 0.41 +/- 0.14 and 0.37 +/- 0.10 respectively). The poor prognostic influence of 11q23 rearrangements seemed to be restricted to infants and older children (> 10 years), who differed significantly from children aged 1-10 years in this regard (P < 0. 01). Patients with t(9;22)-positive ALL seemed to benefit from allogeneic bone marrow transplantation in first remission (P = 0.05). The pEFS for children with t(1;19)(q23;p13)-positive ALL was intermediate (0.63 +/- 0.17), with a tendency to a better outcome for patients with the unbalanced variant der(19)t(1;19). Hyperdiploid ALL patients, subdivided into moderate hyperdiploidy (47-51 chromosomes), massive hyperdiploidy (52-60 chromosomes) and cases in the tri-/tetraploid range (> 60 chromosomes) had the best outcome in the last treatment period (pEFS of 0.81 +/- 0.06, 0.80 +/- 0.04 and 0.88 +/- 0.07 respectively), unless t(1;19), t(8;14), t(9;22) or 11q23 translocations were present. In a multivariate analysis including white blood cell (WBC) count, immunophenotype, age, mediastinal mass, central nervous system involvement and leukaemia karyotype, only WBC and modal chromosome number were shown to be significant independent risk factors (P < 0.01).
在一项基于人群的连续队列研究中，对768例15岁以下急性淋巴细胞白血病（ALL）儿童进行了获得性染色体异常的预后影响评估。该研究队列包括1986年至1997年期间在五个北欧国家（丹麦、芬兰、冰岛、挪威和瑞典）诊断出的所有细胞遗传学异常的儿童ALL病例。整个队列的无事件生存率（pEFS）为0.72±0.02。比较1986年7月至1991年12月和1992年1月至1997年12月这两个治疗期，后一时期的生存率更高（pEFS分别为0.69±0.02和0.76±0.02，P = 0.05）。染色体数少于45条的亚二倍体、t(9;22)(q34;q11)和11q23易位在整个研究期间与预后不良相关（pEFS分别为0.57±0.12、0.41±0.14和0.37±0.10）。11q23重排的不良预后影响似乎仅限于婴儿和大龄儿童（>10岁），在这方面他们与1至10岁的儿童有显著差异（P < 0.01）。t(9;22)阳性的ALL患者在首次缓解期接受异基因骨髓移植似乎有益（P = 0.05）。t(1;19)(q23;p13)阳性的ALL儿童的pEFS处于中等水平（0.63±0.17），不平衡变异型der(19)t(1;19)的患者预后有改善趋势。超二倍体ALL患者分为中度超二倍体（47 - 51条染色体）、大量超二倍体（52 - 60条染色体）和三倍体/四倍体范围（>60条染色体）的病例，在最后一个治疗期预后最佳（pEFS分别为0.81±0.06、0.80±0.04和0.88±0.07），除非存在t(1;19)、t(8;14)、t(9;22)或11q23易位。在一项多变量分析中，纳入了白细胞（WBC）计数、免疫表型、年龄、纵隔肿块、中枢神经系统受累情况和白血病核型，结果显示只有WBC计数和染色体众数是显著的独立危险因素（P < 0.01）。
