Han Jee Yeon, Suh Jin Kyung, Lee Seong Wook, Koh Kyung-Nam, Im Ho Joon, Seo Jong Jin
Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
Blood Res. 2014 Dec;49(4):246-52. doi: 10.5045/br.2014.49.4.246. Epub 2014 Dec 23.
Anaplastic large cell lymphoma (ALCL) is uncommon in children, accounting for approximately 15% of all cases of childhood non-Hodgkin lymphoma. Despite many studies attempting new treatment strategies, treatment outcomes have not significantly improved, and the optimal treatment for pediatric ALCL has not been established.
The records of newly diagnosed ALCL patients at our institute between July 1998 and April 2013 were reviewed. We evaluated the general characteristics of the patients, chemotherapy regimens, overall survival (OS) rates, and event-free survival (EFS) rates.
Twenty-eight ALCL patients were eligible. The median age at diagnosis was 10.8 years. Lymph node involvement was the most common presentation (79%). CCG-5941, a multi-agent T-cell lineage chemotherapy, was the predominant treatment regimen (57%). The five-year OS and EFS rates were 88% and 69%, respectively. Stage, the presence of B symptoms, lung involvement, and bone marrow involvement were significant prognostic factors for EFS (P=0.02, 0.01, 0.01, and 0.02, respectively). Eight patients relapsed, and three died during the study period. Four of the eight patients who relapsed were treated with high-dose chemotherapy and autologous stem cell transplantation (HDCT-ASCT). Two of the four who had undergone HDCT-ASCT developed secondary relapses and were subsequently treated with allogeneic SCT or brentuximab.
We found that treatment outcomes with multi-agent chemotherapy in children with ALCL were similar to those of previous reports, and that relapsed patients could be salvaged with HDCT-ASCT or allogeneic SCT. A prospective, larger cohort study is warranted to define the optimal treatment for pediatric ALCL.
间变性大细胞淋巴瘤（ALCL）在儿童中并不常见，约占儿童非霍奇金淋巴瘤所有病例的15%。尽管许多研究尝试了新的治疗策略，但治疗结果并未显著改善，小儿ALCL的最佳治疗方案尚未确立。
回顾了1998年7月至2013年4月期间我院新诊断的ALCL患者的记录。我们评估了患者的一般特征、化疗方案、总生存率（OS）和无事件生存率（EFS）。
28例ALCL患者符合条件。诊断时的中位年龄为10.8岁。淋巴结受累是最常见的表现（79%）。多药T细胞谱系化疗方案CCG-5941是主要的治疗方案（57%）。五年OS率和EFS率分别为88%和69%。分期、B症状的存在、肺部受累和骨髓受累是EFS的重要预后因素（P分别为0.02、0.01、0.01和0.02）。8例患者复发，3例在研究期间死亡。8例复发患者中有4例接受了大剂量化疗和自体干细胞移植（HDCT-ASCT）。接受HDCT-ASCT的4例患者中有2例出现二次复发，随后接受了异基因干细胞移植或brentuximab治疗。
我们发现，多药化疗治疗小儿ALCL的结果与以往报道相似，复发患者可通过HDCT-ASCT或异基因干细胞移植挽救。有必要进行一项前瞻性、更大队列的研究来确定小儿ALCL的最佳治疗方案。
