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[儿童急性淋巴细胞白血病核型特征与治疗结果的相关性]

[Correlation between karyotypic characteristics and treatment outcome in childhood acute lymphoblastic leukemia].

作者信息

Tie Li-jun, Gu Long-jun, Chen Jing, Dong Lu, Chen Jing, Pan Ci, Ye Hui, Xue Hui-liang, Tang Jing-yan, Wang Yao-ping

机构信息

Department of Hematology/Oncology, Affiliated XinHua Hospital, Shanghai Children's Medical Center, Medical College of Shanghai Jiaotong University, Shanghai 200127, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2006 May;27(5):339-43.

Abstract

OBJECTIVE

To analyze the relationship between karyotypic characteristics and treatment outcome of childhood acute lymphoblastic leukemia (ALL) and compare the difference in karyotypic aberration between ALL patients in China and in western countries.

METHODS

From January 1998 to May 2003, 193 patients with newly diagnosed ALL were enrolled on protocol ALL-XH-99. The patients were classified into 4 groups according to the karyotype of the leukemia cells: normal karyotype, hypodiploid, hyperdiploid and pseudodiploid. Event-free survival (EFS) was estimated by Kaplan-Meier analysis and the distributions of EFS were compared using the log-rank test. A Cox proportional hazards model was used to identify independent prognostic factors.

RESULTS

(1) Of 193 ALL patients, 115 had cytogenetic data. There were 53 (46.09%) with normal karyotype, 29 (25.22%) hyperdiploid, 9 (7.83%) hypodiploid, 4 coexpression of hypodiploid/hyperdiploid and 20 (17.39%) pseudodiploid. The probability of 5-year EFS for the four subgroups were (78.28 +/- 6.34)%, (86.07 +/- 6.47)%, (53.85 +/- 13.83)% and (40.10 +/- 12.17)%, respectively (P = 0.0041). (2) The clinical presentation and early response to treatment had no difference among the four groups, but the events are significantly different. (3) The probability of 5-year EFS for the combined hypodiploid group and the non-hypodiploid group was (53.85 +/- 13.83)% and (69.98 +/- 5.94)%, respectively (P = 0.1281). (4) The probability of 4-year EFS was significantly worse for patients with Philadelphia chromosome than for no Philadelphia chromosome patients [(28.57 +/- 17.07)% vs (70.85 +/- 5.60)%, P = 0.0009]. (5) Multivariate analysis suggested that the karyotypic characteristics, Philadelphia chromosome, age < 1-year or > 12-year, and white blood cell counts were independent prognostic factors.

CONCLUSIONS

The cytogenetic pattern of Chinese childhood ALL patients was similar to that of western countries. Cytogenetic findings especially Philadelphia chromosome had important prognostic significance.

摘要

目的

分析儿童急性淋巴细胞白血病(ALL)的核型特征与治疗结局之间的关系,并比较中国与西方国家ALL患者核型畸变的差异。

方法

1998年1月至2003年5月,193例新诊断的ALL患者纳入ALL-XH-99方案。根据白血病细胞核型将患者分为4组:正常核型、亚二倍体、超二倍体和假二倍体。采用Kaplan-Meier分析估计无事件生存期(EFS),并使用对数秩检验比较EFS的分布。采用Cox比例风险模型确定独立的预后因素。

结果

(1)193例ALL患者中,115例有细胞遗传学数据。正常核型53例(46.09%),超二倍体29例(25.22%),亚二倍体9例(7.83%),亚二倍体/超二倍体共表达4例,假二倍体20例(17.39%)。四个亚组的5年EFS概率分别为((78.28±6.34)%、(86.07±6.47)%、(53.85±13.83)%和(40.10±12.17)%(P = 0.0041)。(2)四组患者的临床表现和早期治疗反应无差异,但事件有显著差异。(3)亚二倍体组合并非亚二倍体组的5年EFS概率分别为(53.85±13.83)%和(69.98±5.94)%(P = 0.1281)。(4)有费城染色体的患者4年EFS概率显著低于无费城染色体的患者[(28.57±17.07)%对(70.85±5.60)%,P = 0.0009]。(5)多因素分析表明,核型特征、费城染色体、年龄<1岁或>12岁以及白细胞计数是独立的预后因素。

结论

中国儿童ALL患者的细胞遗传学模式与西方国家相似。细胞遗传学结果尤其是费城染色体具有重要的预后意义。

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