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34例儿童霍奇金淋巴瘤的临床特征及化疗联合受累部位低剂量放疗的疗效

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

作者信息

Duan Yan-long, Zhang Yong-hong, Jin Ling, Yang Jing, Zhang Rui, Zhou Chun-ju

机构信息

Hematological Center, Capital Medical University, Beijing 100045, China.

出版信息

Zhonghua Er Ke Za Zhi. 2010 Sep;48(9):698-702.

Abstract

OBJECTIVE

To summarize the clinical features and to evaluate outcomes and to assess therapeutic effects 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 Hodgkin 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.

摘要

目的

总结34例在中国接受风险适应性联合化疗及低剂量受累野放射治疗(IFRT)的儿童及青少年霍奇金淋巴瘤患者的临床特征,评估其转归及治疗效果。

方法

2003年1月至2009年4月,我院34例住院的霍奇金淋巴瘤患儿纳入BCH-HL 2003方案(修订的CCG 5942)。病理样本进行集中复审,并根据世界卫生组织指南进行分类。分期基于临床评估,采用Ann Arbor分期系统定义。34例患者根据不同风险因素分为三个治疗组(标准风险、中风险和高风险),接受风险适应性联合化疗及IFRT。所有分析均通过统计软件SPSS进行计算。

结果

34例霍奇金淋巴瘤患者中,男性28例,女性6例。诊断时的中位年龄为8.7岁(范围4岁至15岁)。临床表现方面,53%有大包块淋巴结,47.1%累及4个以上淋巴结区域,44%初诊时有“B”症状。疾病分期分布为:Ⅰ期0%,Ⅱ期21%,Ⅲ期35%,Ⅳ期44%。所有患者均为经典组织学类型,包括三种不同亚型:混合细胞型22例(64.7%)。25例病理样本中有EB病毒编码RNA(EBER)或潜伏膜蛋白(LMP)染色阳性。总生存率(OS)为100%,5年无事件生存率为94.1%,中位随访时间为(26.1±16.3)个月。2例患者在治疗结束后早期复发。器官毒性仅限于血液学Ⅲ级和Ⅳ级,发生率分别为40%和71%。

结论

本研究中的儿童霍奇金淋巴瘤在男性学龄儿童中更为常见。采用风险适应性化疗联合放疗的综合治疗有效且耐受性良好。总体预后良好。

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