《儿童、青少年和青年霍奇金淋巴瘤患者的临床特征和治疗结局:KPHOG 淋巴瘤工作组的一项多中心回顾性研究》。

Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma: a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study.

机构信息

Department of Pediatrics, Yeungnam University College of Medicine, Daegu, Korea.

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

出版信息

J Korean Med Sci. 2020 Nov 30;35(46):e393. doi: 10.3346/jkms.2020.35.e393.

Abstract

BACKGROUND

Hodgkin's lymphoma (HL) constitutes 10%-20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea.

METHODS

We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016.

RESULTS

A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype. Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively ( = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, high-risk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level. In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively.

CONCLUSION

This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

摘要

背景

霍奇金淋巴瘤(HL)约占所有恶性淋巴瘤的 10%-20%,其治愈率较高(5 年生存率约为 90%)。最近,人们对预防长期生存者的继发性并发症(继发性癌症、内分泌紊乱)越来越感兴趣。我们旨在研究韩国儿童、青少年和年轻成人 HL 的流行病学特征和治疗结果。

方法

我们对 2007 年 1 月至 2016 年 8 月期间在韩国 22 家参与机构诊断为 HL 的 224 名年龄<25 岁的患者进行了一项多中心回顾性研究。

结果

男性的诊断年龄较小,比例较高。结节性硬化型 HL 组织病理学亚型最为常见,其次是混合细胞型亚型。81 例(36.2%)、101 例(45.1%)和 42 例(18.8%)患者分别归入低、中、高危组。多柔比星、博来霉素、长春新碱、达卡巴嗪是最常见的方案(n=102,45.5%)。无事件生存率为 86.0%±2.4%,5 年总生存率(OS)为 96.1%±1.4%:低、中、高危组分别为 98.7%±1.3%、97.7%±1.6%和 86.5%±5.6%(=0.021)。B 症状、IV 期疾病、高危、脾累及、结外淋巴瘤和乳酸脱氢酶水平升高的患者 5 年 OS 更差。多变量分析显示,B 症状和结外累及是 OS 不良的预后因素。内分泌紊乱和继发性恶性肿瘤的迟发性并发症分别在 17 例和 6 例患者中观察到。

结论

这是韩国儿童、青少年和年轻成人 HL 流行病学和治疗结果的首次研究。未来需要前瞻性研究,以制定出在儿童、青少年和年轻成人 HL 患者中最大限度降低治疗毒性并最大限度提高治愈率的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d34c/7707923/cf1c0cc94201/jkms-35-e393-g001.jpg

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