Rastogi Archana, Gulati Natasha, Bihari Chhagan, Chaudhary Ashok, Bansal Kalpana, Sasturkar Shridhar, Thapar Shalini, Pamecha Viniyendra
From the Department of Pathology, Institute of Liver and Biliary Sciences, Delhi, India.
Exp Clin Transplant. 2019 Jun;17(3):414-417. doi: 10.6002/ect.2016.0242. Epub 2017 Aug 12.
Progressive multifocal leukoencephalopathy caused by polyoma JC virus is a rare and severe demyelinating disease of the central nervous system. It occurs in immunocompromised patients and is scarcely reported in liver transplant recipients. Brain biopsy demonstrating demyelination with presence of foamy macrophages, relative preservation of axons, astrogliosis, and typical polyomavirus inclusions in the enlarged oligodendroglial nuclei is essential for diagnosis. Here, we report a case of JC virus-associated progressive multifocal leukoencephalopathy in a living-donor liver transplant recipient who was transplanted for hepatitis C virus-related decompensated cirrhosis. Brain biopsy with immunohistochemistry confirmed the diagnosis of progressive multifocal leukoencephalopathy secondary to JC virus. JC virus related-progressive multifocal leukoencephalopathy is associated with extremely poor prognosis. Awareness and consideration of this entity in liver transplant recipients who present with sudden progressive neurologic manifestations can help in prompt diagnosis and timely treatment.
由多瘤病毒 JC 引起的进行性多灶性白质脑病是一种罕见且严重的中枢神经系统脱髓鞘疾病。它发生于免疫功能低下的患者，在肝移植受者中鲜有报道。脑活检显示存在泡沫状巨噬细胞的脱髓鞘、轴突相对保留、星形胶质细胞增生以及在增大的少突胶质细胞核中出现典型的多瘤病毒包涵体对于诊断至关重要。在此，我们报告一例在活体供肝肝移植受者中发生的与 JC 病毒相关的进行性多灶性白质脑病病例，该受者因丙型肝炎病毒相关失代偿性肝硬化接受了肝移植。经免疫组织化学检查的脑活检确诊为继发于 JC 病毒的进行性多灶性白质脑病。JC 病毒相关的进行性多灶性白质脑病预后极差。对于出现突发进行性神经症状的肝移植受者，认识并考虑到这一疾病实体有助于及时诊断和治疗。
