儿童霍奇金淋巴瘤83例临床研究

目的 探讨儿童霍奇金淋巴瘤(HL)的病理及临床特点,初步分析患者EB病毒感染状态,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及相关不良反应.方法 回顾性分析2003年1月至2013年3月接受规范化治疗的83例HL患儿的临床资料.全部行活组织检查病理形态及免疫组织化学检查,参照世界卫生组织(WHO)2001病理分型标准诊断,按HL Ann Arbor分期标准分期.根据不同的危险因素及对治疗的反应,将患儿分成低危、中危、高危3个治疗组.结果 83例患儿中,男性69例,女性14例;Ⅲ～Ⅳ期59例(71.1％).1例为结节性淋巴细胞为主型,其余82例均为经典型HL,其中64例(77.1％)为经典混合细胞型.侵犯部位广泛,巨大瘤块34例(41.0％),大于4个淋巴结区受累26例,有B组症状35例.70例行EB病毒感染指标潜伏膜蛋白(LMP)和(或)EB病毒编码RNA(EBER)染色]检测,65例(92.9％)阳性.随访时间(72±32)个月.低危组3例,中危组27例,高危组53例.总体生存率97.5％,5年无事件生存率92.8％.结论 儿童HL男性多于女性,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 study of 83 cases of pediatric Hodgkin lymphoma

Objective To explore the clinical and pathological features of children with Hodgkin lymphoma (HL) and EBV status,and to summarize the efficacy and toxicity of risk-adapted combination of chemotherapy and low-dose involved-field radiation therapy (IFRT) according to the risk stratification.Methods 83 children with HL from January 2003 to April 2013 were enrolled,who received the detection of biopsy pathological morphology and immunohistochemistry.Pathology samples were reviewed and classified based on the World Health Organization (WHO) guidelines.Staging was based on clinical evaluation and was defined by the Ann Arbor staging system.According to the different risk factors,83 patients were divided into three groups:low-risk group,intermediate-risk group and high-risk group.Results 83 HL children included 69 males and 14 females.59 cases (71.1％) were in stage Ⅲ-Ⅳ.Among 83 patients,one case suffered nodular lymphocyte predominant subtype,other 82 patients had classical HL including 64 cases (77.1％) of mixed cellularity.Tumor invasion was extensive,34 cases (41.0 ％) had bulky lymph nodes,26 cases (31.3 ％) had more than 4 node regions involved,and 35 ceses (42.2 ％) had B symptoms at presentation.70 cases of pathology samples were detected by EBV encoded RNA (EBER) and/or latent membrane protein (LMP) staining,and 65 cases (92.9 ％) were positive.There were 3 cases in low-risk group,27 cases in intermediate-risk group and 53 cases in high-risk group.The overall survival (OS) rate was 97.5 ％and the 5-year event free survival (EFS) rate was 92.8 ％ with a median follow-up of (72±32) months.Conclusions Pediatric HL occurs more frequent in male than in female,which is correlated with EBV and is consist mostly of mixed cellularity.Combined-modality therapy using risk-adapted chemotherapy with radiation is effective and well tolerated,with good prognosis,however,the adverse reaction needs to be observed further.