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一名具有对类固醇有反应的桥脑周围血管增强性慢性淋巴细胞性炎症临床特征（CLIPPERS）的患者出现复发性爱泼斯坦-巴尔病毒阳性（EBV+）原发性中枢神经系统淋巴瘤（PCNSL）

Recurrent Epstein-Barr Virus-positive (EBV+) Primary Central Nervous System Lymphoma (PCNSL) in a Patient with Clinical Features of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS).

作者信息

Nagano Masahiro, Ayaki Takashi, Koita Natsuko, Kitano Toshiyuki, Nishikori Momoko, Goda Naoki, Minamiguchi Sachiko, Ikeda Akio, Takaori-Kondo Akifumi, Takahashi Ryosuke

机构信息

Department of Neurology, Kyoto University Graduate School of Medicine, Japan.

Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2019;58(6):849-854. doi: 10.2169/internalmedicine.1246-18. Epub 2019 Mar 15.

DOI:10.2169/internalmedicine.1246-18
PMID:30880301
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6465023/
Abstract

Primary central nervous system lymphoma (PCNSL) and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) can share clinical features and may be indistinguishable, even after brain biopsy. We encountered a case of Epstein-Barr virus-positive (EBV+) PCNSL recurrence in a patient with clinical features of CLIPPERS, and repeat brain biopsy was required to reach the correct diagnosis. Four years after the initial diagnosis and treatment of PCNSL, "peppering" punctate enhanced lesions with transient steroid responsiveness were detected during brain magnetic resonance imaging (MRI). A second brain biopsy supported a diagnosis of CLIPPERS, while a third biopsy confirmed the diagnosis of recurrent PCNSL.

摘要

原发性中枢神经系统淋巴瘤（PCNSL）和对类固醇有反应的桥脑周围血管增强慢性淋巴细胞性炎症（CLIPPERS）可能具有共同的临床特征，甚至在脑活检后也可能难以区分。我们遇到了一例具有CLIPPERS临床特征的患者，其为爱泼斯坦-巴尔病毒阳性（EBV+）PCNSL复发，需要重复脑活检才能得出正确诊断。在PCNSL首次诊断和治疗四年后，脑部磁共振成像（MRI）检测到“点状”点状强化病变，对类固醇有短暂反应。第二次脑活检支持CLIPPERS的诊断，而第三次活检证实为复发性PCNSL。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/bb3c1ddb1073/1349-7235-58-0849-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/b8644569a7c5/1349-7235-58-0849-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/1ae713d82943/1349-7235-58-0849-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/bd5907431822/1349-7235-58-0849-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/bb3c1ddb1073/1349-7235-58-0849-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/b8644569a7c5/1349-7235-58-0849-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/1ae713d82943/1349-7235-58-0849-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/bd5907431822/1349-7235-58-0849-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e9/6465023/bb3c1ddb1073/1349-7235-58-0849-g004.jpg

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