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

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.