Sutcliffe M J, Shuster J J, Sather H N, Camitta B M, Pullen J, Schultz K R, Borowitz M J, Gaynon P S, Carroll A J, Heerema N A
Department of Pathology, All Children's Hospital, St Petersburg, FL, USA.
Leukemia. 2005 May;19(5):734-40. doi: 10.1038/sj.leu.2403673.
Chromosome aberrations have a major role in pediatric acute lymphoblastic leukemia (ALL) risk assignment. The Children's Cancer Group (CCG) and the Pediatric Oncology Group (POG) independently assessed the significance of trisomy for chromosomes 4, 10, and 17 in National Cancer Institute (NCI) Standard- and High-Risk ALL. Data from 1582 (CCG) and 3902 (POG) patients were analyzed. Eight-year event-free survivals (EFS) of 91% (CCG) and 89% (POG) (P < 0.001) were achieved in patients assigned to NCI Standard Risk whose leukemic cells had simultaneous trisomies 4, 10, and 17. Both groups showed the degree of favorable prognostic importance increased with the actual number of favorable trisomies. POG analyses also demonstrated hyperdiploidy (> or =53 chromosomes) was less of an independently significant prognostic factor in the absence of these key trisomies. This finding supported conclusions from previous CCG and POG studies that specific trisomies are more important than chromosome number in predicting outcome in pediatric B-precursor ALL. In NCI Higher Risk patients, the number of favorable trisomies was not prognostically significant, but showed the same trend. Moreover, specific trisomies 4, 10, and 17 remain associated with favorable prognosis in Standard-Risk B-precursor ALL, even in the context of very different treatment approaches between the groups.
染色体畸变在小儿急性淋巴细胞白血病(ALL)风险评估中起主要作用。儿童癌症研究组(CCG)和小儿肿瘤研究组(POG)分别评估了4号、10号和17号染色体三体在国立癌症研究所(NCI)标准风险和高风险ALL中的意义。分析了来自1582例(CCG)和3902例(POG)患者的数据。白血病细胞同时存在4号、10号和17号染色体三体的NCI标准风险患者的8年无事件生存率(EFS)分别为91%(CCG)和89%(POG)(P<0.001)。两组均显示,有利三体的实际数量越多,预后的有利程度越高。POG分析还表明,在没有这些关键三体的情况下,超二倍体(≥53条染色体)作为独立的显著预后因素的作用较小。这一发现支持了CCG和POG先前研究的结论,即在预测小儿B前体ALL的预后时,特定的三体比染色体数量更重要。在NCI高风险患者中,有利三体的数量在预后方面不具有显著意义,但显示出相同的趋势。此外,即使两组的治疗方法差异很大,特定的4号、10号和17号染色体三体在标准风险B前体ALL中仍与良好预后相关。