Paediatric Haematology-Oncology Program, Research Centre, Instituto Nacional de Câncer, Rio de Janeiro, Brazil.
Br J Haematol. 2013 Apr;161(2):224-36. doi: 10.1111/bjh.12250. Epub 2013 Feb 21.
Acute leukaemia in early childhood - and mainly infant leukaemia (IL) - is characterized by acquired genetic alterations, most commonly by the presence of distinct MLL rearrangements (MLL-r). The aim of this study was to investigate possible correlations between clinical features and molecular analyses of a series of 545 childhood leukaemia (≤24 months of age) cases: 385 acute lymphoblastic leukaemia (ALL) and 160 acute myeloid leukaemia (AML). The location of the genomic breakpoints was determined in a subset of 30 MLL-r cases. The overall survival of the investigated cohort was 60·5%, as determined by the Kaplan-Meier method. Worse outcomes were associated with age at diagnosis ≤6 months (P < 0·001), high white blood cell count (P = 0·001), and MLL-r (P = 0·002) in ALL, while children with AML displayed a poorer outcome (P = 0·009) regardless of their age strata. Moreover, we present first evidence that MLL-r patients with poor outcome preferentially displayed chromosomal breakpoints within MLL intron 11. Based on the literature, most MLL-r IL display a breakpoint localization towards intron 11, which in turn may explain their worse clinical course. In summary, the MLL breakpoint localization is of clinical importance and should be considered as a novel outcome predictor for MLL-r patients.
儿童急性白血病 - 主要是婴儿白血病(IL) - 的特征是获得性遗传改变,最常见的是存在明显的 MLL 重排(MLL-r)。本研究的目的是调查 545 例儿童白血病(≤24 个月龄)病例系列的临床特征与分子分析之间的可能相关性:385 例急性淋巴细胞白血病(ALL)和 160 例急性髓细胞白血病(AML)。在 30 例 MLL-r 病例的亚组中确定了基因组断裂点的位置。通过 Kaplan-Meier 方法确定了研究队列的总生存率为 60.5%。较差的结果与诊断时的年龄≤6 个月(P<0.001)、高白细胞计数(P=0.001)和 ALL 中的 MLL-r(P=0.002)相关,而 AML 患儿无论年龄分层如何预后均较差(P=0.009)。此外,我们首次提供了证据表明预后不良的 MLL-r 患者优先显示 MLL 内含子 11 内的染色体断裂点。根据文献,大多数 MLL-r IL 显示出向内含子 11 定位的断裂点,这反过来又可能解释了它们较差的临床病程。总之,MLL 断裂点定位具有临床重要性,应被视为 MLL-r 患者的新预后预测因子。