[Diagnosis and treatment of anaplastic large-cell lymphoma in children and adolescents: a retrospective multicenter survey study].

Objective: To provide a descriptive review and improve our understanding of the clinical characteristics and treatment outcome of pediatric anaplastic large cell lymphoma (ALCL) in China. Method: The clinical data and outcomes of patients under 16 years of age with newly histopathologically-confirmed ALCL in 10 large single institutions in China between January 2009 and June 2014, were retrospectively analyzed.The event-free survival (EFS) was analyzed by the Kaplan-Meier method.The risk factors of disease progression or relapse were evaluated by logistic regression analysis.Significance was defined as P<0.05. Result: Of the 80 eligible patients (52 male, 28 female), the median age was 8.4 years (range, 1.3-15.7 years). Two patients (3%) were Stage Ⅰ, 9(11%) Stage Ⅱ, 64 (80 %) Stage Ⅲ, and 5(6%) Stage Ⅳ.The median time of follow-up was 25.2 months (range, 7.1-74.8 months), 55 patients survived without disease at the last of follow-up, the 3-year EFS was (65±6)%. Sixty-five patients (81%) were treated with the Chinese Children Cancer Group-B cell-non-Hodgkin Lymphoma 2010 protocol, regimen and 15 cases (19%) were treated with other regimens.The 3-year EFS were (68±5)% vs.(65±20)% (P=0.21). The 3-year EFS was (57±7)% and (78±11)% for patients with or without B symptoms (P=0.01). Twenty-four patients experienced disease progression or relapse. The median time from initial diagnosis to tumor failure was 7.0 months (ranged, 1.5-42.6 months) (median). At the last evaluation, there were 5 patients still alive after disease progression and relapse. By univariate analysis, sex (P=0.04) and B symptoms (P=0.00) were identified as risk factors of disease progression or relapse.Nevertheless, only B symptoms (HR 5.60, 95%CI 1.47-21.27, P≤0.05) were risk factors in multivariate analysis. Conclusion: The presenting features of children and adolescents with ALCL and efficiency in this study were similar to those reported by western countries.Refinement of therapeutic strategies to improve survival for patients with disease progression or relapse should be the priority in future clinical study.目的：了解儿童间变大细胞淋巴瘤(ALCL)的临床特点和诊治现状。 方法：多中心回顾性研究。收集2009年1月至2014年6月中国抗癌协会小儿肿瘤专业委员会和中华医学会儿科学分会血液学组10个儿童血液肿瘤中心收治的病理诊断明确且年龄≤16岁的ALCL病例，随访终点截至2015年6月30日。以邮件通讯方式完成资料采集。Kaplan－Meier方法计算无事件生存率(EFS)，组间的差异型行Log－rank检验，多因素Logistic回归分析疾病进展或复发危险因素。 结果： 80例来自10个三甲医院儿童血液肿瘤中心的ALCL患儿被纳入研究，男52例、女28例，中位年龄8.4(1.3～15.7)岁。临床分期(St Jude儿童和青少年非霍奇金淋巴瘤分期系统)：Ⅰ期2例(3%)，Ⅱ期9例(11%)，Ⅲ期64例(80%)，Ⅳ期5例(6%)。中位随访时间25.2(7.1～74.8)个月，55例在末次随访时无病生存，3年EFS为(65±6)%。65例应用中国儿童肿瘤协作组－B细胞非霍奇金淋巴瘤－2010化疗(CCCG－BNHL－2010)方案，15例应用其他治疗方案，患儿的3年EFS分别为(68±5)%、(65±20)%。52例伴体重减轻和(或)发热和(或)盗汗的B组症状和28例不伴B组症状患儿的3年EFS分别为(57±7)%和(78±11)%(P＝0.01)。24例患儿出现疾病进展或复发，从诊断到疾病进展或复发的中位时间为7.0(1.5 ～ 42.6)个月。疾病进展或复发后，仅5例于末次随访时存活。单因素分析显示，性别(P＝0.04)和伴B组症状(P＝0.00)是ALCL患儿疾病复发或进展的危险因素；多因素分析仅伴有B组症状与疾病进展或复发有关(风险比5.60，95%可信区间1.47～21.27，P≤0.05)。 结论： 10个儿童血液肿瘤中心的ALCL患儿临床特点和初治疗效基本符合目前国际上对于此病的普遍认识。提高对疾病进展或复发患儿的救治能力是下一期方案更新的重点。.