Choi Ho Jung, Shin Juhee, Kang Sunghan, Suh Jin Kyung, Kim Hyery, Koh Kyung-Nam, Im Ho Joon
Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Blood Res. 2020 Dec 31;55(4):262-274. doi: 10.5045/br.2020.2020220.
Lymphoblastic lymphoma (LBL) is the second most common subtype of pediatric non-Hodgkin lymphoma. Modified treatments derived from the LSA2-L2 regimen resulted in encouraging survival, but toxicities and long-term sequelae have been problematic. At present, the acute lymphoblastic leukemia (ALL)-type protocol has demonstrated efficacy in LBL. We analyzed the outcomes of children and adolescents with LBL treated with various regimens.
From 1991‒2018, this study enrolled 63 patients diagnosed with LBL at Asan Medical Center. Medical records were retrospectively analyzed.
Among 63 patients, most patients (38.1%) presented with stage IV at diagnosis, and two had central nervous system (CNS) involvement. At a median follow-up of 160 months, the 5-year event free survival (EFS), overall survival (OS), and relapse free survival (RFS) were 68.8%, 79.3%, and 71.3%, respectively. Among 61 patients who received chemotherapy, 27 patients (44.3%) received the NY protocol, and 14 (23.0%) received the ALL-type protocol. There was no significant difference in 5-yr OS (85.2%/78.6%), EFS (73.5%/78.6%), and RFS (73.5%/78.6%) between the NY and ALL protocol groups, regardless of immunophenotype. Thirteen patients (21.3%) received prophylactic cranial radiotherapy with no difference in the incidence of CNS relapse based on irradiation.
This study showed no difference in outcome between the NY and ALL-type protocols, regardless of stage or immunophenotype. In addition to improving the effectiveness of treatment, it is necessary to continuously appraise the appropriate chemotherapy regimen, considering toxicities and long-term prognosis, for pediatric LBL.
淋巴母细胞淋巴瘤（LBL）是儿童非霍奇金淋巴瘤的第二常见亚型。源自LSA2-L2方案的改良治疗取得了令人鼓舞的生存率，但毒性和长期后遗症一直是个问题。目前，急性淋巴细胞白血病（ALL）型方案已在LBL中显示出疗效。我们分析了接受各种方案治疗的儿童和青少年LBL患者的结局。
1991年至2018年，本研究纳入了63例在峨山医学中心诊断为LBL的患者。对病历进行回顾性分析。
63例患者中，大多数患者（38.1%）在诊断时处于IV期，2例有中枢神经系统（CNS）受累。中位随访160个月时，5年无事件生存率（EFS）、总生存率（OS）和无复发生存率（RFS）分别为68.8%、79.3%和71.3%。在61例接受化疗的患者中，27例（44.3%）接受了NY方案，14例（23.0%）接受了ALL型方案。NY方案组和ALL方案组之间的5年OS（85.2%/78.6%）、EFS（73.5%/78.6%）和RFS（73.5%/78.6%）无显著差异，无论免疫表型如何。13例患者（21.3%）接受了预防性颅脑放疗，基于放疗的CNS复发发生率无差异。
本研究表明，无论分期或免疫表型如何，NY方案和ALL型方案的结局无差异。除了提高治疗效果外，还需要持续评估合适的化疗方案，同时考虑毒性和长期预后，以治疗儿童LBL。
