当代治疗背景下儿童急性淋巴细胞白血病中t(1;19)易位的临床意义：儿童癌症研究组报告

Clinical significance of translocation t(1;19) in childhood acute lymphoblastic leukemia in the context of contemporary therapies: a report from the Children's Cancer Group.

作者信息

Uckun F M, Sensel M G, Sather H N, Gaynon P S, Arthur D C, Lange B J, Steinherz P G, Kraft P, Hutchinson R, Nachman J B, Reaman G H, Heerema N A

机构信息

Children's Cancer Group ALL Biology Reference Laboratory and Wayne Hughes Institute, St. Paul, MN 55113, USA.

出版信息

J Clin Oncol. 1998 Feb;16(2):527-35. doi: 10.1200/JCO.1998.16.2.527.

DOI:10.1200/JCO.1998.16.2.527

PMID:9469337

Abstract

PURPOSE

The nonrandom translocation t(1;19) has been associated with poor outcome in pediatric B-lineage acute lymphoblastic leukemia (ALL). Because most patients treated by contemporary therapies now achieve improved outcomes, we have reassessed the prognostic significance of t(1;19).

PATIENTS AND METHODS

Cytogenetic data were accepted for 1,322 children (<21 years old) with newly diagnosed ALL enrolled between 1988 and 1994 on risk-adjusted studies of the Children's Cancer Group (CCG). Forty-seven patients (3.6%) were t(1;19) positive (+); 1,275 (96.4%) were t(1;19) negative (-). Clinical characteristics and treatment outcome were compared using standard methods.

RESULTS

Translocation (1;19)+ patients were more likely than t(1;19)- patients to be 10 years of age or greater (P < .001) or CD10+ CD19+ CD34- (P < .0001), or nonwhite (P = .02). Patients with a balanced t(1;19) were less likely to be hyperdiploid than patients with an unbalanced der(19)t(1;19). Event-free survival (EFS) was similar for the overall group of t(1;19)+ and t(1;19)- patients, with 4-year estimates of 69.5% (SD, 6.8%) and 74.8% (SD, 1.3%; P = .48), respectively. However, patients with unbalanced der(19)t(1;19) had significantly better outcomes than patients with balanced t(1;19): 4-year EFS were 80.6% (SD, 7.1%) and 41.7% (SD, 13.5%), respectively (P = .003). These differences were maintained within the individual studies analyses and after exclusion of t(1;19)+ patients whose cells were hyperdiploid with more than 50 chromosomes.

CONCLUSION

The overall group of t(1;19)+ patients, as well as the subgroup with an unbalanced der(19)+ (1;19) had outcomes similar to that of t(1;19)- patients, whereas patients with balanced t(1;19) had poorer outcomes. Thus, although the overall prognostic significance of t(1;19) has been obviated by contemporary risk-adjusted protocols, the balanced t(1;19) translocation remains an adverse prognostic factor.

摘要

目的

非随机易位t(1;19)与儿童B系急性淋巴细胞白血病（ALL）的不良预后相关。由于现在大多数接受当代疗法治疗的患者预后得到改善，我们重新评估了t(1;19)的预后意义。

患者与方法

接受了1988年至1994年间纳入儿童癌症组（CCG）风险调整研究的1322例新诊断ALL儿童（<21岁）的细胞遗传学数据。47例患者（3.6%）t(1;19)阳性（+）；1275例（96.4%）t(1;19)阴性（-）。采用标准方法比较临床特征和治疗结果。

结果

与t(1;19)-患者相比，t(1;19)+患者更有可能年龄在10岁及以上（P<.001）或CD10+CD19+CD34-（P<.0001），或非白人（P=.02）。与具有不平衡der(19)t(1;19)的患者相比，具有平衡t(1;19)的患者超二倍体的可能性较小。t(1;19)+和t(1;19)-患者总体组的无事件生存率（EFS）相似，4年估计值分别为69.5%（标准差，6.8%）和74.8%（标准差，1.3%；P=.48）。然而，具有不平衡der(19)t(1;19)的患者比具有平衡t(1;19)的患者预后明显更好：4年EFS分别为80.6%（标准差，7.1%）和41.7%（标准差，13.5%）（P=.003）。这些差异在各个研究分析中以及排除细胞超二倍体且染色体数超过50条的t(1;19)+患者后仍然存在。