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伪装为神经系统脱髓鞘疾病的血管内大B细胞淋巴瘤1例

Intravascular large B-cell lymphoma disguised as a demyelinating disease of the nervous system:a case report
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摘要 血管内大B细胞淋巴瘤(intravascular large B-cell lymphoma,IVLBCL)是一种罕见且具高度侵袭性的结外非霍奇金淋巴瘤。本文报道1例IVLBCL患者,以中枢神经系统症状起病,诊治过程曲折漫长,曾诊断为中枢神经系统脱髓鞘病变和血管炎,历经糖皮质激素及免疫抑制剂治疗失败、先后2次手术病理活检才最终确诊为IVLBCL。确诊后采用大剂量甲氨蝶呤联合R-CHOP方案和R-DHAP方案先后各治疗4个疗程和6个疗程,病灶缩小,症状改善,但神经功能始终未恢复,生活无法自理,目前规律随访中。 Intravascular large B-cell lymphoma(IVLBCL)is a rare and highly aggressive extranodal non-Hodgkin lymphoma.A case of IVLBCL who initially presented with central nervous system symptoms was reported.His diagnosis and treatment process were long and challenging,during which he was initially diagnosed with central nervous system demyelinating disease and vasculitis.After failed treatments with corticosteroids and immunosuppressants,the diagnosis was ultimately confirmed through two separate surgical pathological biopsies.Following diagnosis,he underwent four cycles of high-dose methotrexate combined with R-CHOP therapy,followed by six cycles of R-DHAP therapy.Although imaging showed lesion regression and symptoms improved,neurological function had been never recovered.He remains unable to perform daily activities independently and is currently under regular follow-up.
作者 陈深 马晶晶 林之光 顾洁 黄延焱 陈宏 陈波斌 CHEN Shen;MA Jingjing;LIN Zhiguang;GU Jie;HUANG Yanyan;CHEN Hong;CHEN Bobin(Department of Hematology,Huashan Hospital,Fudan University,Shanghai 200040,China;Department of General Practice,Huashan Hospital,Fudan University,Shanghai 200040,China;Department of Pathology,Huashan Hospital,Fudan University,Shanghai 200040,China)
出处 《上海医药》 2025年第6期11-15,共5页 Shanghai Medical & Pharmaceutical Journal
关键词 血管内大B 细胞淋巴瘤 中枢神经系统 脑活检 神经影像 intravascular large B-cell lymphoma central nervous system brain biopsy neuroimaging
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