实体器官移植相关进行性多灶性脑白质病诊治进展  

Advances of diagnosis and treatment solid organ transplantation associated progressive multifocal leukoencephalopathy

作　　者：徐培航 王璐琳[1] 蔡宇航 练巧燕 王晓华 徐鑫[1] 何建行[1] 巨春蓉[1] Xu Peihang;Wang Lulin;Cai Yuhang;Lian Qiaoyan;Wang Xiaohua;Xu Xin;He Jianxing;Ju Chunrong(First Affiliated Hospital,Guangzhou Medical University,Guangzhou Institute of Respiratory Health,National Center for Respiratory Medicine,State Key Laboratory of Respiratory Disease,Guangzhou 510120,China)

机构地区：[1]广州医科大学附属第一医院广州呼吸健康研究院,国家呼吸医学中心,国家呼吸疾病临床研究中心,呼吸疾病国家重点实验室,广州510120

出　　处：《中华器官移植杂志》2023年第9期568-573,共6页Chinese Journal of Organ Transplantation

基　　金：广东省自然科学基金(2022A1515012216);广东省钟南山医学基金会(ZNSA-2020013);呼吸疾病国家重点实验室/广州呼吸健康研究院/国家呼吸中心临床自主探索项目(SKLRHQN20205);广州呼吸健康研究院基础研究计划(202201020371)。

摘　　要：进行性多灶性脑白质病(progressive multifocal leukoencephalopathy,PML)是一种由JC病毒感染引起的罕见但严重的中枢神经系统疾病,主要发生在包括实体器官移植(solid organ transplant,SOT)受者在内的免疫抑制人群。SOT相关的PML的常见临床症状为认知行为障碍。其病理表现为颅内多灶性脱髓鞘病变,神经影像学常显示为顶枕叶区域的脑白质病变。临床诊断通常需要结合临床表现、颅脑核磁共振以及脑脊液JC病毒的检测。治疗方面,目前缺乏特异性药物,主要依靠支持性治疗和免疫抑制剂减量等免疫调控策略。PML的预后不佳,早期诊断和增强适应性免疫应答是SOT受者PML管理的关键。Progressive multifocal leukoencephalopathy(PML)is a rare and yet serious central nervous system disorder due to JC viral infection.PML occurs predominantly in immunocompromised individuals,including solid organ transplant(SOT)recipients.Clinically,SOT-related PML commonly presents as cognitive and behavioral impairments.Pathologically,PML is characterized by multifocal demyelinating lesions,with neuroimaging technique typically revealing white matter damage in the temporoparietal regions.Clinical diagnosis usually involves integrating clinical manifestations,cranial magnetic resonance imaging,and detection of JC virus in cerebrospinal fluid.Currently,specific medications for PML are lacking,and the treatment mainly relies on supportive care and immunomodulatory strategies.The prognosis of PML remains unfavorable,early diagnosis and enhanced adaptive immune responses are crucial for PML management in SOT recipients.

关 键 词：进行性多灶性脑白质病 器官移植 免疫治疗 中枢神经系统脱髓鞘 

分 类 号：R741[医药卫生—神经病学与精神病学]