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儿童近四倍体 T 细胞急性淋巴细胞白血病:AIEOP-BFM ALL 研究结果。

Near-tetraploid T-cell acute lymphoblastic leukaemia in childhood: Results of the AIEOP-BFM ALL studies.

机构信息

Pediatric Hematology-Oncology Unit, Division of Pediatrics, Woman-Mother-Child Department, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Lausanne, Switzerland.

Pediatric Hemato-Oncology Center, Fondazione MBBM, University Milano-Bicocca, Ospedale San Gerardo-Monza, Italy.

出版信息

Eur J Cancer. 2022 Nov;175:120-124. doi: 10.1016/j.ejca.2022.08.013. Epub 2022 Sep 13.

Abstract

BACKGROUND

Near-tetraploidy-defined by DNA index 1.79-2.28 or 81-103 chromosomes-is a rare cytogenetic abnormality observed both in children and adults with T-cell acute lymphoblastic leukaemia (T-ALL) and its prognostic value is not yet determined.

PATIENTS AND METHODS

We report a retrospective study conducted in paediatric patients with newly diagnosed T-ALL treated in AIEOP-BFM ALL 2000 and 2009 studies. 31 near-tetraploid T-ALL patients (1.4%) are compared to T-ALL patients without near-tetraploidy.

RESULTS

Near-tetraploid karyotype was associated with lower frequency of high-risk features: white blood cells count at diagnosis ≥100,000/μL (19.3% versus 41.0%, p-value < 0.001), PPR (13.3% versus 35.8%, p-value = 0.01) and minimal residual disease high-risk at the end of consolidation phase Induction B (4.03% versus 14.6%, p-value = 0.001). Complete remission was achieved at the end of induction phase (day 33) in 100% near-tetraploid T-ALL patients, compared to 93.2% T-ALL without near-tetraploidy.

CONCLUSION

Overall, we found that near-tetraploid T-ALL in newly diagnosed paediatric patients is associated with low-risk presenting features, with favourable treatment response and outcome.

摘要

背景

近四倍体(定义为 DNA 指数 1.79-2.28 或 81-103 条染色体)是一种罕见的细胞遗传学异常,在儿童和成人 T 细胞急性淋巴细胞白血病(T-ALL)中均有观察到,但其预后价值尚未确定。

患者和方法

我们报告了一项在接受 AIEOP-BFM ALL 2000 和 2009 研究治疗的新诊断为 T-ALL 的儿科患者中进行的回顾性研究。将 31 例近四倍体 T-ALL 患者(1.4%)与无近四倍体的 T-ALL 患者进行比较。

结果

近四倍体核型与较低的高危特征频率相关:诊断时白细胞计数≥100,000/μL(19.3%比 41.0%,p 值<0.001)、PPR(13.3%比 35.8%,p 值=0.01)和巩固期 B 诱导结束时高风险微小残留病(4.03%比 14.6%,p 值=0.001)。近四倍体 T-ALL 患者在诱导期结束时(第 33 天)达到完全缓解的比例为 100%,而无近四倍体的 T-ALL 患者为 93.2%。

结论

总的来说,我们发现新诊断的儿科患者中近四倍体 T-ALL 与低危表现特征相关,治疗反应和结局良好。

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