Pontocerebellar Progressive Multifocal Leukoencephalopathy. Radiological, Clinical, Histological and Immunohistochemical Findings in a Hematological Patient

J. Ortiz, J. Paniagua, Isidro Cavaco, R. Rivas, Andrea Jiménez, Luis Chinchilla, E. Muñoz, M. Ludeña
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Abstract

Objective: To describe the radiological, histological and immunohistochemical findings in a case of Progressive Multifocal Leukoencephalopathy (PML) affecting the cerebellar peduncles in a patient with chronic lymphocytic leukemia. Patient and Methods: Magnetic Resonance Imaging (MRI), histological picture (H.E., Kluver-Barrera) and immunohistochemical picture (GFAP, neurofilaments, CD68, JC virus) were obtained. Results: 1) Magnetic resonance imaging: Asymmetric and progressive lesions on middle cerebellar pe-duncles, that were hyperintense in T2/FLAIR, extended towards the pons, had no mass effect and were unmodified after intravenous contrast. 2) Histology: Marked reactive gliosis with cytopathic changes suggesting viral infection, plus demyelination areas with axonal preservation. 3) Immunohistochemistry: Marked positivity for viral (polyoma and JC virus) markers in glial cells showing cytopathic changes. Conclusions: The importance of histological and immunohistochemical diagnosis in everyday assistance; of the collaboration between clinicians, radiologists and pathologists; and the validity of post-mortem studies as a key element for research and clinical quality assessment must be stressed.
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桥小脑进行性多灶性脑白质病。血液病患者的放射学、临床、组织学和免疫组织化学表现
目的:描述1例慢性淋巴细胞白血病累及小脑蒂的进行性多灶性脑白质病(PML)的影像学、组织学和免疫组化表现。患者与方法:磁共振成像(MRI)、组织学图(H.E、Kluver-Barrera)、免疫组化图(GFAP、神经丝、CD68、JC病毒)。结果:1)磁共振成像:小脑中小丘不对称、进行性病变,T2/FLAIR呈高信号,向脑桥延伸,无肿块效应,静脉造影剂未修饰。2)组织学:反应性胶质瘤明显,细胞病变提示病毒感染,同时伴有轴突保存的脱髓鞘区。3)免疫组化:胶质细胞中病毒(多瘤和JC病毒)标记物明显阳性,显示细胞病变。结论:组织学和免疫组织化学诊断在日常辅助中的重要性;临床医生,放射科医生和病理学家之间的合作;必须重视死后研究的有效性,将其作为研究和临床质量评估的关键因素。
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