Jeha S, Pei D, Raimondi S C, Onciu M, Campana D, Cheng C, Sandlund J T, Ribeiro R C, Rubnitz J E, Howard S C, Downing J R, Evans W E, Relling M V, Pui C-H
Department of Oncology, St Jude Children's Research Hospital, Memphis, TN 38105, USA.
Leukemia. 2009 Aug;23(8):1406-9. doi: 10.1038/leu.2009.42. Epub 2009 Mar 12.
To evaluate the impact of contemporary therapy on the clinical outcome of children with pre-B acute lymphoblastic leukemia (ALL) and the t(1;19)/TCF3/PBX1, we analyzed 735 patients with B-cell precursor ALL treated in four successive protocols at St Jude Children's Research Hospital. The 41 patients with the t(1;19) had a comparable event-free survival to that of the 694 patients with other B-cell precursor ALL (P=0.63; 84.2+/-7.1% (s.e.) vs 84.0+/-1.8% at 5 years). However, patients with the t(1;19) had a lower cumulative incidence of any hematological relapse (P=0.06; 0 vs 8.3+/-1.2% at 5 years) but a significantly higher incidence of central nervous system (CNS) relapse (P<0.001; 9.0+/-5.1% vs 1.0+/-0.4% at 5 years). In a multivariate analysis, the t(1;19) was an independent risk factor for isolated CNS relapse. These data suggest that with contemporary treatment, patients with the t(1;19) and TCF3/PBX1 fusion have a favorable overall outcome but increased risk of CNS relapse.
为评估当代疗法对伴t(1;19)/TCF3/PBX1的前B细胞急性淋巴细胞白血病（ALL）患儿临床结局的影响，我们分析了在圣裘德儿童研究医院接受四个连续方案治疗的735例B细胞前体ALL患者。41例伴有t(1;19)的患者与694例其他B细胞前体ALL患者的无事件生存率相当（P = 0.63；5年时为84.2±7.1%（标准误）对84.0±1.8%）。然而，伴有t(1;19)的患者任何血液学复发的累积发生率较低（P = 0.06；5年时为0对8.3±1.2%），但中枢神经系统（CNS）复发的发生率显著较高（P<0.001；5年时为9.0±5.1%对1.0±0.4%）。在多变量分析中，t(1;19)是孤立性CNS复发的独立危险因素。这些数据表明，采用当代治疗方法，伴有t(1;19)和TCF3/PBX1融合的患者总体结局良好，但CNS复发风险增加。
