首页|15例儿童非免疫缺陷原发中枢神经系统淋巴瘤的临床研究

15例儿童非免疫缺陷原发中枢神经系统淋巴瘤的临床研究

Clinical study of 15 cases of primary non-immunodeficient central nervous system lymphoma in children

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回顾性分析首都医科大学附属北京儿童医院2013年5月至2023年5月收治年龄≤18岁的15例非免疫缺陷原发中枢神经系统淋巴瘤(PCNSL)患儿的临床资料,总结临床特点以及基于病理组织亚型的、以大剂量甲氨蝶呤(HD-MTX)为基础的化疗方案的治疗效果.男女比例2.7∶1,中位确诊年龄7.2(2.3~16.4)岁,临床首发症状以颅高压伴颅神经受损为主,影像学以多发病灶为主.免疫功能正常的儿童PCNSL发病率极低,以HD-MTX为基础的化疗整体预后良好,部分病情评估稳定者可减量维持或不予维持治疗.初治以非HD-MTX为主的化疗,病情进展或复发患儿再次予HD-MTX为基础的化疗仍有效.
Clinical data of 15 primary central nervous system lymphoma(PCNSL)children aged≤18 years admitted to our hospital between May 2013 to May 2023 were retrospectively analyzed.Our goal was to summarize the clinical features of children and investigate the therapeutic effect of a high-dose methotrexate(HD-MTX)based chemotherapy regimen on this disease.The male-to-female ratio was 2.7:1,and the median age was 7.2(2.3-16.4)years at diagnosis.The initial clinical symptoms were primarily cranial hypertension,with imaging findings revealing multiple lesions.Pediatric PCNSL with normal immune function has a favorable prognosis with HD-MTX-based chemotherapy.Patients with a stable disease can be treated with minimal or no maintenance.HD-MTX-based chemotherapy remains effective when the disease progresses or recurs after an initial course of non-HD-MTX-based chemotherapy.

高慧霞、张宁宁、周春菊、金玲、杨菁、黄爽、张梦、李楠、张永红、段彦龙

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国家儿童医学中心,首都医科大学附属北京儿童医院儿童肿瘤中心肿瘤内科,儿童血液病与肿瘤分子分型北京市重点实验室,儿童肿瘤国家临床重点专科,儿科重大疾病研究教育部重点实验室,北京 100045

首都医科大学附属北京儿童医院影像中心,北京 100045

首都医科大学附属北京儿童医院病理科,北京 100045

2024

中华血液学杂志
中华医学会

中华血液学杂志

CSTPCD北大核心
影响因子:1.17
ISSN:0253-2727
年,卷(期):2024.45(2)
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