An autopsy case of progressive multifocal leukoencephalopathy with massive iron deposition in juxtacortical lesions
An autopsy case of progressive multifocal leukoencephalopathy with massive iron deposition in juxtacortical lesions
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DOI：
10.1111/neup.12898
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发表时间：
2023-02-27
期刊：
影响因子：
2.3
通讯作者：
Itoh，Yoshiaki
中科院分区：
文献类型：
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作者：
Okamoto，Kosuke;Takeda，Akitoshi;Itoh，Yoshiaki
进行性多灶性白质脑病（PML）是由JC病毒感染少突胶质细胞引起的严重脱髓鞘疾病。关于PML患者铁沉积的报道很少。在此，我们报告了一例PML，患者为一名71岁女性，在接受利妥昔单抗联合环磷酰胺、多柔比星、长春新碱和泼尼松龙治疗滤泡性淋巴瘤16个月后，出现双侧视力障碍和进行性失语。磁共振成像显示左顶叶和其他脑叶的白色病变，并在前皮质病变中有大量铁沉积。JC病毒PCR检测呈阳性，证实PML诊断。尽管接受了甲氟喹和米氮平治疗，但患者在6个月后死亡。尸检时，发现脱髓鞘主要发生在左顶叶。此外，富含含铁血黄素的巨噬细胞和含铁蛋白的反应性星形胶质细胞在邻近白色病变的前皮质区域中丰富。这是一个以前未报告的淋巴瘤后PML病例，其中铁沉积被证实的放射学和病理学。
Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease caused by JC virus infection of oligodendrocytes. Little has been reported on iron deposits in patients with PML. Herein, we report a case of PML with massive iron deposition in the juxtacortical regions attaching white matter lesions in a 71‐year‐old woman who developed bilateral visual disturbance and progressive aphasia after 16 months of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone treatment for follicular lymphoma. Magnetic resonance imaging revealed white matter lesions in the left parietal and other lobes with massive iron deposition in the juxtacortical lesions. A PCR test for JC virus was positive, confirming the diagnosis of PML. Despite treatment with mefloquine and mirtazapine, the patient died six months later. At autopsy, demyelination was found dominantly in the left parietal lobe. Moreover, hemosiderin‐laden macrophages and reactive astrocytes containing ferritin were abundant in the juxtacortical regions adjacent to the white matter lesions. This is a previously unreported case of PML after lymphoma, in which iron deposition was confirmed both radiologically and pathologically.