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来自五个儿科急性髓细胞白血病(AML)治疗方案的伴有 -5/5q- 异常的儿科患者的结局:来自儿童肿瘤协作组的报告。

Outcome of pediatric patients with acute myeloid leukemia (AML) and -5/5q- abnormalities from five pediatric AML treatment protocols: a report from the Children's Oncology Group.

机构信息

Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, Ottawa, Canada.

出版信息

Pediatr Blood Cancer. 2013 Dec;60(12):2073-8. doi: 10.1002/pbc.24573. Epub 2013 Aug 30.

Abstract

BACKGROUND

Abnormalities of chromosome 5q (-5/5q-) are associated with poor prognosis in adults with acute myeloid leukemia (AML). However, there are no large studies on outcomes of children with -5/5q- AML. To determine the disease correlates of this group, we retrospectively analyzed cytogenetic data from five studies of childhood AML.

PROCEDURE

Data from patients whose cytogenetic clones included -5/5q-, with the exception of those with acute promyelocytic leukemia or Down syndrome, were included.

RESULTS

Of the 2,240 patients with cytogenetic data available, 26 (1.2%) had -5 or 5q-. A significant number of these patients were age 11-21 (61.5%, P = 0.031) and had M0 morphology compared with patients without -5/5q- (24.0% vs. 2.8%, P < 0.001). Twenty-two of the 26 patients had a complete remission (CR) response to induction chemotherapy. The 5-year overall survival (OS) from the time of diagnosis for the -5/5q- patients was significantly lower than for patients without -5/5q- (27 ± 17% vs. 50 ± 2%, P = 0.027). Similarly, from induction CR, patients with -5/5q- had significantly worse disease free survival, OS and relapse risk than those without this abnormality (27 ± 19% vs. 46 ± 2%, P = 0.035, 32 ± 20% vs. 57 ± 2%, P = 0.025, 68 ± 21% vs. 45 ± 2%, P = 0.01, respectively).

CONCLUSIONS

Pediatric patients with AML and -5/5q- had a very poor outcome. These findings support the need for new or novel therapies for these patients.

摘要

背景

染色体 5q 异常(-5/5q-)与成人急性髓系白血病(AML)的预后不良有关。然而,目前尚无关于儿童 -5/5q-AML 结局的大型研究。为了确定这一组的疾病相关性,我们回顾性分析了五项儿童 AML 研究的细胞遗传学数据。

方法

纳入细胞遗传学克隆中包括 -5/5q-的患者数据,但不包括急性早幼粒细胞白血病或唐氏综合征患者。

结果

在可获得细胞遗传学数据的 2240 例患者中,有 26 例(1.2%)存在 -5 或 5q-。与无 -5/5q-的患者相比,这些患者中有相当一部分年龄在 11-21 岁(61.5%,P=0.031),且具有 M0 形态(24.0% vs. 2.8%,P<0.001)。26 例患者中有 22 例对诱导化疗有完全缓解(CR)反应。-5/5q-患者从诊断到总生存(OS)的 5 年生存率明显低于无 -5/5q-的患者(27±17% vs. 50±2%,P=0.027)。同样,从诱导 CR 来看,与无这种异常的患者相比,-5/5q-患者的无病生存、OS 和复发风险显著更差(27±19% vs. 46±2%,P=0.035,32±20% vs. 57±2%,P=0.025,68±21% vs. 45±2%,P=0.01)。

结论

患有 AML 和 -5/5q-的儿科患者预后极差。这些发现支持为这些患者提供新的或新型治疗方法的必要性。

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