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根据 AML-BFM 98 方案,对大量接受统一治疗的儿童急性髓系白血病患者中特定染色体异常的预后影响。

Prognostic impact of specific chromosomal aberrations in a large group of pediatric patients with acute myeloid leukemia treated uniformly according to trial AML-BFM 98.

机构信息

Pediatric Hematology and Oncology, Medical School Hannover, Carl-Neuberg-Strasse 1, Hannover, Germany.

出版信息

J Clin Oncol. 2010 Jun 1;28(16):2682-9. doi: 10.1200/JCO.2009.25.6321. Epub 2010 May 3.

Abstract

PURPOSE

Because cytogenetic data are essential for risk stratification of childhood acute myeloid leukemia (AML), the impact of chromosomal aberrations is crucial.

PATIENTS AND METHODS

Data of a large group of patients younger than 18 years treated according to study AML-Berlin-Frankfurt-Münster (BFM) 98 (n = 454), including their cytogenetics, were analyzed.

RESULTS

The favorable outcome in the subgroups of patients with t(8;21), inv(16), and t(15;17), with an overall survival of 91% (SE, 4%), 92% (SE, 6%), and 87% (SE, 5%), respectively, was confirmed. Within this group, the 5-year probability of event-free survival (pEFS) of all 17 children with t(8;21) and additional aberrations apart from del(9q) or -X/-Y was 100%. As expected, the cytogenetic finding of a complex karyotype (n = 35; pEFS, 33%; SE, 8%) or a monosomy 7 (n = 12; pEFS, 17%; SE, 11%) was associated with a poor outcome. Compared with remaining patients with cytogenetic data (pEFS, 48%; SE, 2%), prognosis in patients with an MLL rearrangement (n = 91) was inferior (pEFS, 34%; SE, 5%; P = .0005). Particularly, children with t(9;11) and additional aberrations (n = 13; pEFS, 31%; SE, 14%) and MLL rearrangements other than t(9;11) and t(11;19) (n = 41; pEFS, 24%; SE, 7%) had an unfavorable outcome. Nine patients with aberrations in 12p showed an adverse prognosis (pEFS, 11%; SE, 10%). The outcome of patients with aberrations of chromosome 5 (n = 13) was better than expected (pEFS, 50%; SE, 13%).

CONCLUSION

Because the prognostic value of rare recurrent chromosomal aberrations still has to be elucidated, these data will contribute to future risk stratification for the treatment of pediatric AML.

摘要

目的

由于细胞遗传学数据对于儿童急性髓系白血病(AML)的风险分层至关重要,因此染色体异常的影响至关重要。

患者和方法

对根据 AML-Berlin-Frankfurt-Münster(BFM)98 方案治疗的大量年龄小于 18 岁的患者(n = 454)的细胞遗传学数据进行了分析。

结果

在 t(8;21)、inv(16)和 t(15;17)患者亚组中,证实了良好的结局,总生存率分别为 91%(SE,4%)、92%(SE,6%)和 87%(SE,5%)。在这一组中,17 名 t(8;21)患者和除 del(9q)或 -X/-Y 之外的其他异常的 5 年无事件生存概率(pEFS)为 100%。如预期的那样,复杂核型(n = 35;pEFS,33%;SE,8%)或单体 7(n = 12;pEFS,17%;SE,11%)的细胞遗传学发现与不良结局相关。与具有细胞遗传学数据的其余患者(pEFS,48%;SE,2%)相比,MLL 重排患者(n = 91)的预后较差(pEFS,34%;SE,5%;P =.0005)。特别是,t(9;11)和其他异常(n = 13;pEFS,31%;SE,14%)以及除 t(9;11)和 t(11;19)以外的 MLL 重排患者(n = 41;pEFS,24%;SE,7%)的预后不良。9 名 12p 异常患者预后不良(pEFS,11%;SE,10%)。5 号染色体异常患者(n = 13)的预后好于预期(pEFS,50%;SE,13%)。

结论

由于罕见的复发性染色体异常的预后价值仍有待阐明,这些数据将有助于未来儿童 AML 治疗的风险分层。

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