Jeon Woojung, Koh Young Kwon, Kang Sunghan, 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. 2022 Mar 31;57(1):41-50. doi: 10.5045/br.2021.2021164.
Aggressive mature B-cell non-Hodgkin lymphoma (B-NHL) is the most common non-Hodgkin lymphoma in children. The outcome of chemotherapy for B-NHL has improved over decades.
We reviewed 82 children and adolescents with B-NHL diagnosed at Asan Medical Center between 1993 and 2020. The D-COMP/COMP (daunomycin-cyclophosphamide, doxorubicin, vincristine, and prednisolone), Pediatric Oncology Group (POG)-9219/9315/9317, R-CHOP/CHOP (rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone), and Lymphomes Malins B 89 (LMB89)/LMB96 regimens were administered. In 2018, rituximab was added to the LMB protocol (R-LMB) for advanced-staged Burkitt lymphoma (BL). The patients' clinical features and treatment outcomes were retrospectively analyzed.
The most common subtype was BL (61%), followed by diffuse large B-cell lymphoma (DLBCL) (35%). The median age was 7.8 (range, 1.3‒16.4) years, and the most frequently used regimen was French‒American‒British (FAB)/LMB96 (58 patients, 70.7%). The 5-year overall survival (OS) and event-free survival (EFS) rates were 92.5% and 85.7%, respectively. The EFS rates of patients with BL and DLBCL were 90.0% and 79.3%, respectively. Among the FAB/LMB risk groups, group C (85.7%) had a significantly lower 5-year OS ( =0.037). Eleven events occurred (6 relapses, 3 deaths, and 2 secondary malignancies) during the median follow-up of 7.1 (range, 3.7‒118.5) months. Two patients treated with R-LMB had good outcomes without complications.
Various treatment regimens have favorable outcomes in pediatric patients with B-NHL. However, further studies are needed to improve survival in high-risk patients. In addition, careful monitoring for acute toxicity or secondary malignancy due to intensive multidrug chemotherapy is required.
侵袭性成熟B细胞非霍奇金淋巴瘤（B-NHL）是儿童中最常见的非霍奇金淋巴瘤。几十年来，B-NHL的化疗效果有所改善。
我们回顾了1993年至2020年在峨山医学中心诊断为B-NHL的82名儿童和青少年。采用了D-COMP/COMP（柔红霉素-环磷酰胺、阿霉素、长春新碱和泼尼松龙）、儿童肿瘤学组（POG）-9219/9315/9317、R-CHOP/CHOP（利妥昔单抗-环磷酰胺、阿霉素、长春新碱和泼尼松龙）以及淋巴瘤恶性B 89（LMB89）/LMB96方案。2018年，利妥昔单抗被添加到晚期伯基特淋巴瘤（BL）的LMB方案（R-LMB）中。对患者的临床特征和治疗结果进行了回顾性分析。
最常见的亚型是BL（61%），其次是弥漫性大B细胞淋巴瘤（DLBCL）（35%）。中位年龄为7.8岁（范围1.3 - 16.4岁），最常用的方案是法国-美国-英国（FAB）/LMB96（58例患者，70.7%）。5年总生存率（OS）和无事件生存率（EFS）分别为92.5%和85.7%。BL和DLBCL患者的EFS率分别为90.0%和79.3%。在FAB/LMB风险组中，C组（85.7%）的5年OS显著较低（P = 0.037）。在中位随访7.1个月（范围3.7 - 118.5个月）期间发生了11起事件（6例复发、3例死亡和2例继发性恶性肿瘤）。两名接受R-LMB治疗的患者预后良好，无并发症。
各种治疗方案对儿童B-NHL患者都有良好的疗效。然而需要进一步研究以提高高危患者的生存率。此外，需要密切监测强化多药化疗引起的急性毒性或继发性恶性肿瘤。
