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

作　　者：高慧霞 张宁宁[2] 周春菊[3] 金玲[1] 杨菁[1] 黄爽[1] 张梦[1] 李楠[1] 张永红[1] 段彦龙[1] Gao Huixia;Zhang Ningning;Zhou Chunju;Jin Ling;Yang Jing;Huang Shuang;Zhang Meng;Li Nan;Zhang Yonghong;Duan Yanlong(Medical Oncology Department,Pediatric Oncology Center,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing Key Laboratory of Pediatric Hematology Oncology,National Key Clinical Discipline of Pediatric Oncology,Key Laboratory of Major Diseases in Children,Ministry of Education,Beijing 100045,China;Department of Imaging,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China;Department of Pathology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]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 [2]首都医科大学附属北京儿童医院影像中心,北京100045 [3]首都医科大学附属北京儿童医院病理科,北京100045

出　　处：《中华血液学杂志》2024年第2期190-194,共5页Chinese Journal of Hematology

摘　　要：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.

关 键 词：大剂量甲氨蝶呤 非免疫缺陷 预后良好 多发病灶 病情评估 维持治疗 颅高压 病情进展 

分 类 号：R739.4[医药卫生—肿瘤]