Choeyprasert Worawut, Anurathapan Usanarat, Pakakasama Samart, Sirachainan Nongnuch, Songdej Duantida, Lertthammakiat Surapong, Hongeng Suradej
Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Pediatr Int. 2019 Jan;61(1):49-57. doi: 10.1111/ped.13739.
In the modern era of chemotherapy, the outcome of pediatric non-Hodgkin lymphoma (NHL) continues to improve internationally. Limited data such as information on epidemiology and survival, however, are available in Asian countries.
Children (≤15 years old) diagnosed with histologically proven NHL from 1998 to 2014 were retrospectively analyzed.
In total, 114 patients were enrolled; they were predominantly male (65.8%) and had advanced disease (stage III, IV; 71.9%). Of these, 22.8% had Burkitt lymphoma, 20.2% had diffuse large B-cell lymphoma, 21.1% had lymphoblastic lymphoma, 20.2% had large cell lymphoma, and 15.8% had peripheral T-cell lymphoma. Twenty-nine patients died, especially of uncontrolled disease (62.1%) and infection (20.7%). During a median follow up of 78.4 months, Kaplan-Meier 5 year event-free and overall survival rates were 71.5% ± 4.3% and 74.8% ± 4.1%, respectively, regardless of subtype. B symptoms (i.e. systemic symptoms of fever, night sweats, and weight loss that can be associated with both Hodgkin's lymphoma and non-Hodgkin's lymphoma) and advanced disease had a significant negative impact on 5 year survival. No other prognostic factor was found, but survival tended to have a negative correlation with age.
Pediatric NHL is aggressive, with a high prevalence of peripheral T-cell lymphoma. The present treatment stratification seems to be effective compared with that used in developed countries.
在现代化疗时代，小儿非霍奇金淋巴瘤（NHL）的治疗效果在国际上持续改善。然而，亚洲国家关于流行病学和生存率等方面的可用数据有限。
对1998年至2014年诊断为组织学确诊的NHL的15岁及以下儿童进行回顾性分析。
共纳入114例患者；他们以男性为主（65.8%），且多为晚期疾病（III期、IV期；71.9%）。其中，22.8%为伯基特淋巴瘤，20.2%为弥漫性大B细胞淋巴瘤，21.1%为淋巴母细胞淋巴瘤，20.2%为大细胞淋巴瘤，15.8%为外周T细胞淋巴瘤。29例患者死亡，主要死于疾病控制不佳（62.1%）和感染（20.7%）。在中位随访78.4个月期间，无论亚型如何，Kaplan-Meier法计算的5年无事件生存率和总生存率分别为71.5%±4.3%和74.8%±4.1%。B症状（即可能与霍奇金淋巴瘤和非霍奇金淋巴瘤相关的发热、盗汗和体重减轻等全身症状）和晚期疾病对5年生存率有显著负面影响。未发现其他预后因素，但生存率与年龄呈负相关趋势。
小儿NHL具有侵袭性，外周T细胞淋巴瘤患病率高。与发达国家使用的治疗分层相比，目前的治疗分层似乎有效。
