An autopsy case of progressive multifocal leukoencephalopathy with massive iron deposition in juxtacortical lesions.

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Neuropathology Pub Date : 2023-10-01 Epub Date: 2023-02-27 DOI:10.1111/neup.12898
Kosuke Okamoto, Akitoshi Takeda, Hiroyuki Hatsuta, Terunori Sano, Masaki Takao, Masahiko Ohsawa, Yukio Miki, Kazuo Nakamichi, Yoshiaki Itoh
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Abstract

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.

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一例进行性多灶性白质脑病的尸检病例,病变旁有大量铁沉积。
进行性多灶性白质脑病(PML)是由少突胶质细胞感染JC病毒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,该患者在16岁后出现双侧视觉障碍和进行性失语症 数月的利妥昔单抗加环磷酰胺、阿霉素、长春新碱和泼尼松治疗滤泡性淋巴瘤。磁共振成像显示左顶叶和其他叶有白质病变,边缘病变有大量铁沉积。JC病毒的PCR检测呈阳性,证实了PML的诊断。尽管使用了甲氟喹和米氮平治疗,患者仍有6人死亡 几个月后。尸检发现脱髓鞘主要发生在左顶叶。此外,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白质病变附近的边缘区域大量存在。这是一个以前未报道的淋巴瘤后PML病例,其中铁沉积在放射学和病理学上都得到了证实。
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来源期刊
Neuropathology
Neuropathology 医学-病理学
CiteScore
4.10
自引率
4.30%
发文量
105
审稿时长
6-12 weeks
期刊介绍: Neuropathology is an international journal sponsored by the Japanese Society of Neuropathology and publishes peer-reviewed original papers dealing with all aspects of human and experimental neuropathology and related fields of research. The Journal aims to promote the international exchange of results and encourages authors from all countries to submit papers in the following categories: Original Articles, Case Reports, Short Communications, Occasional Reviews, Editorials and Letters to the Editor. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.
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