State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Division of Pediatric Blood Diseases Center, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 288 Nanjing Road, Tianjin, 300020, China.
J Cancer Res Clin Oncol. 2021 Sep;147(9):2775-2788. doi: 10.1007/s00432-021-03551-4. Epub 2021 Mar 2.
Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is rare in China and case reports are varied. We conducted an in-depth analysis of newly diagnosed children with T-ALL from January 1999 to April 2015 in our center, to show the biological differences between Chinese ETP-ALL children and other immune types of T-ALL.
The newly diagnosed children with T-ALL were divided into four groups according to their immunophenotype: ETP-ALL, early non-ETP-ALL, cortical T-ALL and medullary T-ALL. Disease-free survival (DFS), event-free survival (EFS), and overall survival (OS) rates were estimated by the Kaplan-Meier method. The Cox regression model was used for multivariate analysis.
A total of 117 newly diagnosed children with T-ALL were enrolled in this study. The 10-year EFS and OS rates for all patients were 59.0 ± 4.7% and 61.0 ± 4.7%, respectively, with a median follow-up of 64 (5-167) months. Univariate analysis showed that ETP-ALL patients had the lowest 10-year DFS rate of 32.1 ± 11.7%, while cortical T-ALL had the highest DFS rate of 81.3 ± 8.5% compared with early non-ETP-ALL (61.6 ± 7.0%) and medullary T-ALL (59.1 ± 10.6%). Multivariate analysis demonstrated that only ETP-ALL and involvement of the central nervous system were independent prognostic factors.
Compared with other subtypes, pediatric ETP-ALL had a poor treatment response and high recurrence rate while cortical T-ALL appeared to have much better outcome. Our observations highlight the need for an individualized treatment regime for ETP-ALL.
早期 T 细胞前体急性淋巴细胞白血病(ETP-ALL)在中国较为罕见,且病例报道各不相同。本研究对 1999 年 1 月至 2015 年 4 月在我院新诊断的 T-ALL 患儿进行了深入分析,旨在展示中国 ETP-ALL 患儿与其他免疫类型 T-ALL 之间的生物学差异。
根据免疫表型将新诊断的 T-ALL 患儿分为 4 组:ETP-ALL、早期非 ETP-ALL、皮质 T-ALL 和髓质 T-ALL。采用 Kaplan-Meier 法估计无病生存(DFS)、无事件生存(EFS)和总生存(OS)率。采用 Cox 回归模型进行多变量分析。
本研究共纳入 117 例新诊断的 T-ALL 患儿。所有患者的 10 年 EFS 和 OS 率分别为 59.0±4.7%和 61.0±4.7%,中位随访时间为 64(5-167)个月。单因素分析显示,ETP-ALL 患儿的 10 年 DFS 率最低,为 32.1±11.7%,而皮质 T-ALL 的 DFS 率最高,为 81.3±8.5%,明显高于早期非 ETP-ALL(61.6±7.0%)和髓质 T-ALL(59.1±10.6%)。多因素分析表明,仅 ETP-ALL 和中枢神经系统受累是独立的预后因素。
与其他亚型相比,儿童 ETP-ALL 治疗反应差、复发率高,而皮质 T-ALL 似乎预后较好。我们的观察结果强调了为 ETP-ALL 制定个体化治疗方案的必要性。