关键词: ferritin hemosiderin-phagocytic macrophage reactive astrocyte susceptibility-weighted image visual disturbance

Mesh : Female Humans Aged Leukoencephalopathy, Progressive Multifocal / pathology Autopsy Rituximab Cyclophosphamide Lymphoma / pathology Magnetic Resonance Imaging JC Virus Brain / pathology

来  源:   DOI:10.1111/neup.12898

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.
摘要:
进行性多灶性白质脑病(PML)是由少突胶质细胞的JC病毒感染引起的严重脱髓鞘疾病。关于PML患者的铁沉积的报道很少。在这里,我们报告了一例PML病例,在71岁的女性中,在利妥昔单抗加环磷酰胺治疗16个月后出现双侧视觉障碍和进行性失语,并伴有白质病变。阿霉素,长春新碱,和泼尼松龙治疗滤泡性淋巴瘤。磁共振成像显示左顶叶和其他叶的白质病变,并在皮质病变中大量铁沉积。JC病毒的PCR检测呈阳性,确认PML的诊断。尽管使用甲氟喹和米氮平治疗,病人六个月后死亡。尸检时,在左顶叶主要发现脱髓鞘。此外,富含含铁血黄素的巨噬细胞和含有铁蛋白的反应性星形胶质细胞在靠近白质病变的近区丰富。这是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证实。
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