儿童霍奇金淋巴瘤34例临床分析

目的 认识儿童霍奇金淋巴瘤(HL)的病理及临床特点,总结根据危险度分层治疗即化疗联合低剂量受累野放疗的疗效,探讨治疗相关毒性作用.方法 前瞻性研究2003年1月至2009年4月我院治疗的34例霍奇金淋巴瘤患儿;全部行活检病理形态及免疫组化检查,参照世界卫生组织(WHO)2001病理分型标准诊断、按霍奇金淋巴瘤An arbor分期标准分期.根据患儿不同的危险因素及对治疗反应将患儿分成低危、中危、高危3个治疗组.结果 男28例,女6例;以Ⅲ期、Ⅳ期居多.22例为经典混合细胞型(64.7%).侵犯部位广泛;巨大瘤块18例(52.9%);大于4个淋巴结区受累16例;B组症状15例.25例行EB病毒感染指标即潜伏膜蛋白(LMP)或EB病毒编码RNA(EBER)染色,100%阳性.随访时间(26.1±16.3)个月.低危组0例,中危组14例,高危组20例.总体生存率100%、预计5年无事件生存率94.1%;中、高危患儿Ⅲ、Ⅳ度血液学不良反应累积发生率分别为40.0%和70.8%.结论 儿童时期霍奇金淋巴瘤男多于女,与EB病毒相关,病理以混合细胞型居多,预后相对较好,根据疾病危险度进行联合化疗及低剂量受累野放疗方案的近期疗效可靠,但随访时间尚短,需进一步观察远期不良反应.

Clinical characteristics of 34 children with Hodgkin lymphoma and efficacy of treatment with chemotherapy plus low dose radiotherapy on involved sites

Objective To summarize the clinical features and to evaluate outcomes and to assess therapeutic efects in 34 children and adolescents with Hodgkin lymphoma treated with risk-adapted combination chemotherapy and low-dose,involved-field radiation therapy(IFRT)in China Method From January 2003 to April 2009,34 hospitalized children with Hodgkin lymphoma were enrolled into the BCH-HL 2003 protocol(revised CCG 5942)in our hospital Pathological samples were reviewed centrally and classified based on the World Health Organization guidelines.Staging was based on clinical evaluation and was defined by the Ann Arbor staging system.The 34 patients were treated according to the different risk factors in three treatment groups(standard,intermediate,and high risk),and received risk-adapted combination chemotherapy and IFRT.All analyses were calculated by the statistical program SPSS.Result Of the 34 Hodgkin lymphoma patients,28 were male and 6 were female.The median age was 8.7 years (range from 4 years to 15 years)at the time of diagnosis.In terms of clinical presentation,53% had bulky lymph nodes,47.1% had more than 4 node regions involved and 44% had "B" symptoms at presentation.The distribution for stage of disease was 0% for Stage Ⅰ ,21% for Stage Ⅱ,35% for Stage Ⅲ and 44% for Stage Ⅳ disease.All patients had classical histology consisting of three different sub-discipline:22 cases of mixed cellularity(64.7%).In pathological samples of 25 cases there was EBV encoded RNA(EBER)or latent membrane protein(LMP)staining.The overall survival(OS)was 100% and the 5-year event-free survival was 94.1% with a median follow-up of(26.1±16.3)months.Two patients had early relapse after treatment was finished.Organ toxicity was limited to hematological grades Ⅲ and Ⅳ at rates of 40% and 71% respectively.Conclusion Childhood Hodgkdn lymphoma in our study was more frequently seen in male school aged children.Combined-modality therapy using risk-adapted chemotherapy with radiation is effective and well tolerated.The overall prognosis was good.