NHL-BFM Study Center and Department of Pediatric Hematology and Oncology, Justus-Liebig-University, D-35392 Giessen, Germany.
J Clin Oncol. 2011 Aug 1;29(22):3065-71. doi: 10.1200/JCO.2011.34.8417. Epub 2011 Jun 27.
To evaluate risk factors for outcome in children and adolescents with relapse of anaplastic large-cell lymphoma (ALCL) after comparable first-line therapy.
We analyzed a population-based cohort of 74 children with relapsed ALCL after Berlin-Frankfurt-Muenster-type first-line therapy between April 1990 and December 2003. The recommended salvage strategy was reinduction chemotherapy followed by autologous hematopoietic stem-cell transplantation (SCT).
With a median follow-up time of 8.4 years (range, 4.5 to 16.4 years), the 5-year overall survival (OS) rate after first relapse was 57% ± 6%. Survival correlated with time of relapse and clinically advanced dissemination. Five-year OS of 16 patients who experienced progression during first-line therapy was 25% ± 11% compared with 66% ± 6% for 58 patients with a later relapse (P = .002). Five-year OS of 11 patients with bone marrow or CNS involvement was 27% ± 13% compared with 62% ± 6% for 63 patients without involvement (P = .001). Five-year event-free survival (EFS) and OS of 39 children who received the recommended autologous SCT were 59% ± 8% and 77% ± 7%, respectively. EFS after autologous SCT was significantly associated with time to relapse (progression: n = 3; EFS, 0; later relapse: n = 36; EFS, 64% ± 8%; P = .014) and CD3 expression (CD3 negative: n = 25; EFS, 72% ± 9%; CD3 positive: n = 11; EFS, 18% ± 12%; P < .001), but not with site of relapse, conditioning regimen, or graft manipulation. No relapses occurred among 10 patients with relapsed CD3-positive ALCL treated with allogeneic SCT.
Reinduction chemotherapy followed by autologous SCT proved feasible and efficacious for patients with a first relapse of CD3-negative ALCL after first-line therapy. Patients with progression during first-line therapy or relapsed CD3-positive ALCL may benefit from allogeneic SCT.
评估儿童和青少年在接受柏林-法兰克福-明斯特（Berlin-Frankfurt-Muenster，BFM）一线治疗后复发性间变大细胞淋巴瘤（anaplastic large-cell lymphoma，ALCL）的结局相关风险因素。
我们分析了一个基于人群的队列，其中 74 例儿童在 1990 年 4 月至 2003 年 12 月期间接受 BFM 一线治疗后复发间变大细胞淋巴瘤。推荐的挽救性策略是再诱导化疗，随后进行自体造血干细胞移植（hematopoietic stem-cell transplantation，SCT）。
中位随访时间为 8.4 年（范围，4.5 至 16.4 年），首次复发后的 5 年总生存（overall survival，OS）率为 57%±6%。生存与复发时间和临床晚期播散相关。16 例在一线治疗期间进展的患者的 5 年 OS 率为 25%±11%，而 58 例较晚复发的患者为 66%±6%（P=0.002）。11 例骨髓或中枢神经系统受累的患者 5 年 OS 率为 27%±13%，而 63 例无受累的患者为 62%±6%（P=0.001）。接受推荐的自体 SCT 的 39 例儿童的 5 年无事件生存（event-free survival，EFS）和 OS 率分别为 59%±8%和 77%±7%。自体 SCT 后的 EFS 与复发时间（进展：n=3；EFS，0；较晚复发：n=36；EFS，64%±8%；P=0.014）和 CD3 表达（CD3 阴性：n=25；EFS，72%±9%；CD3 阳性：n=11；EFS，18%±12%；P＜0.001）显著相关，但与复发部位、预处理方案或移植物处理无关。10 例接受一线治疗后复发的 CD3 阳性 ALCL 患者接受异基因 SCT 治疗，无一例复发。
对于一线治疗后首次复发的 CD3 阴性 ALCL 患者，再诱导化疗联合自体 SCT 是可行且有效的。一线治疗期间进展或复发的 CD3 阳性 ALCL 患者可能受益于异基因 SCT。
