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目的：分析儿童青少年霍奇金淋巴瘤（HL）患者的病理特征、临床表现及其预后影响因素。方法：收集青岛大学医学院附属医院血液儿科2001 年5 年至2013 年8 月收治的23 例经病理确诊的儿童青少年HL患者的临床资料，采用Fisher 确切概率法等进行各组间差异检验。结果：确诊病例共23 例，中位年龄7.5 岁，男:女发病比例=6.7:1，Ⅰ期、Ⅱ期、Ⅲ期、Ⅳ期分别占13.0%、 26.2%、30.4％、30.4%；结节性淋巴细胞为主型（NLPHL）1 例（4.3%），经典型HL22 例（95.7%）：混合细胞型（MC）10 例(43.5%)，淋巴细胞为主型（LP）11 例（47.9%），结节硬化型（NS）1 例(4.3%)；受累部位以颈部淋巴结最多见，其次依次为纵隔、腹腔及腹膜后、脾、骨骼、肺组织等，其中巨大纵隔肿块者2 例；具有B症状者8 例(34.7％)。化疗2 个疗程评估总有效率为100%，完全缓解（CR）率69.6%，部分缓解（PR）率30.4%。Ⅰ期和Ⅱ期患者CR率100%，明显高于Ⅲ期和Ⅳ期患者（50%），P<0.05；23 例患者7 例复发，复发率Ⅲ期和Ⅳ期患者为46.2%，Ⅰ期和Ⅱ期患者为11.1%，前者高于后者（P=0.0098）；有B 症状与无B 症状患者之间复发率有显著统计学差异（P=0.019）；2 例有巨大包块患者皆复发；各病理分型与疾病的复发间差异无统计学意义（x2=2.695，P>0.05）。结论：儿童霍奇金淋巴瘤预后相对较好，但Ⅲ期和Ⅳ期、合并B症状及大肿块或大纵隔肿瘤的患者复发率高，应依据疾病危险度分层治疗，以期更好的预后。

英文摘要:

Objective:To investigate the pathological characteristic, clinical features and prognosis in children and adolescent with Hodgkin's lymphoma.Methods:23 patients of childhood and adolescent with Hodgkin’s lymphoma were treated in department of pediatric Hematology of the affiliated Hospital of Qingdao university from 2001 to 2013. The clinicopathological and follow-up data of the patients were retrospectively reviewed. The clinical rate in the difference of groups was analyzed by x2 test and fisher exact test.Results:23 cases were diagnosed in the study. The mean age was 7.5 years. The sex-ratio was 6.7:1. Stage I, II, III and IV were accounted for 13.0%, 26.2%, 30.4%, 30.4%respectively. There was 1 patient of NLPHL (4.3%), 10 patients of MC (43.5%), 11 patients of LP (47.9%) and 1 patient of NS (4.3%). The involved parts were mostly the cervical lymph node, followed by mediastinum, enterocoelia, retroperitoneal, spleen, skeleton and lung tissue, of which there were 2 patients with huge mediastinummass. 8 patients had B symptoms (34.7%) at diagnosis. After 2 cycles of initial chemotherapy, the overall response rate (ORR) reached 100%. And complete response (CR) rate was 69.6%while partial response (PR) rate was 30.4%. The CR rate of patients at stage I and II (100%) was significantly higher than that at stage III and IV (50%), P<0.05. 7 of 23 patients were relapsed. The recurrence rate of stage III and IV was higher than that at stage I and II (46.2%verse 11.1%，P=0.0098). For the recurrence rate of patients, there was significantly statistical difference between patients with B symptoms and those without B symptoms (P=0.019). 2 patients with mediastinal neoplasmwere relapsed. There was no statistical difference between the relapse of disease and each histological subtype (x2=2.695, P>0.05).Conclusion:The prognosis of children and adolescent with Hodgkin's lymphoma is better, but the patients of stage III and IV or ones with B symptom and huge mediastinal lump have higher recurrence rate. The hierarchical therapy is adopted for children with HL in order to reach the long-termsurvival.

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