Cytogenetics and prognosis in childhood lymphoblastic leukaemia: results of MRC UKALL X
Cytogenetics and prognosis in childhood lymphoblastic leukaemia: results of MRC UKALL X
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DOI：
10.1046/j.1365-2141.1997.3493163.x
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发表时间：
1997-10-01
影响因子：
6.5
通讯作者：
Richards, SM
中科院分区：
文献类型：
--
作者：
Chessells, JM;Swansbury, GJ;Richards, SM
我们分析了一组502例急性淋巴细胞白血病（ALL）患儿诊断时细胞遗传学检查结果对预后的影响，这些患儿接受MRC UKALL X治疗，诊断时检测到克隆性细胞遗传学异常。尽管与以前的试验相比，接受该方案治疗的儿童的预后总体上有所改善，一些细胞遗传学定义的组仍然与不良结果相关，并且倍性保留了一些预后意义，高超二倍体ALL患者（39%的患者有克隆性异常）有良好的结局，5年无事件生存率为71%。近单倍体（1%）、亚二倍体（9%）和低超二倍体（16.5%）的预后相对较差，5年无事件生存率分别为17%、42%和49%。12例Ph阳性白血病患儿中仅有2例缓解后存活，染色体11 q23异常也与治疗失败的高风险相关。相比之下，t（1;19）与5年无事件生存率87.5%的改善相关。一些其他的非随机异常被确定为没有明确的预后significant.We的结论，某些遗传变化的识别仍然是重要的急性淋巴细胞白血病的管理，虽然分子诊断的临床相关异常，现在是否可以取代细胞遗传学在临床试验的背景下，仍有待确定。
We have analysed the prognostic influence of cytogenetic findings at diagnosis in a group of 502 children with acute lymphoblastic leukaemia (ALL), treated on MRC UKALL X, in whom clonal cytogenetic abnormalities were detected at diagnosis, Despite the overall improvement in outcome in children treated on this protocol compared with previous trials, some cytogenetically-defined groups were still associated with a poor outcome and ploidy retained some prognostic significance, Patients with high hyperdiploid ALL (39% of those with clonal abnormalities) had a favourable outcome with event free survival of 71% at 5 years. Those with near haploidy (1%), hypodiploidy (9%) and low hyperdiploidy (16.5%) had a relatively poor prognosis with event-free survival at 5 years of 17%, 42% and 49% respectively, Only two of 12 children with Ph-positive leukaemia are alive in remission and abnormalities of chromosome 11q23 were also associated with a high risk of treatment failure. In contrast, the t(1;19) was associated with improved event-free survival of 87.5% at 5 years. A number of other non-random abnormalities were identified with no clear prognostic significance.We conclude that identification of certain genetic changes remains important in the management of acute lymphoblastic leukaemia, although whether molecular diagnosis of clinically relevant abnormalities can now supplant cytogenetics in the clinical trials context remains to be determined.