{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T13:32:31Z","timestamp":1789565551126,"version":"build-2803163510"},"reference-count":34,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,7,9]],"date-time":"2023-07-09T00:00:00Z","timestamp":1688860800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:p>A 72\u2010year\u2010old woman with dermatomyositis (DM) developed neurological manifestation, and magnetic resonance imaging (MRI) revealed multiple T2\/fluid\u2010attenuated inversion recovery (FLAIR)\u2010hyperintense lesions predominantly in the deep white matter of the cerebral hemisphere. Punctate or linear contrast enhancement was observed surrounding the T1\u2010hypointense area. Multiple T2\/FLAIR\u2010hyperintense lesions were aligned along with the corona radiata. Malignant lymphoma was first suspected, and a brain biopsy was performed. Pathological investigation suggested the provisional diagnosis of \u201csuspicious of malignant lymphoma.\u201d Owing to emergent clinical conditions, high\u2010dose methotrexate (MTX) therapy was conducted, and then T2\/FLAIR\u2010hyperintense lesions were dramatically reduced. However, the diagnosis of malignant lymphoma was concerning since multiplex PCR demonstrated clonal restriction of the <jats:italic>Ig H<\/jats:italic> gene for B cells and <jats:italic>TCR beta<\/jats:italic> genes for T cells. Histopathology revealed the infiltration of both CD4<jats:sup>+<\/jats:sup> and CD8<jats:sup>+<\/jats:sup> T cells, and the CD4<jats:sup>+<\/jats:sup>\/CD8<jats:sup>+<\/jats:sup> ratio was 4.0. Moreover, prominent plasma cells were observed, in addition to CD20<jats:sup>+<\/jats:sup> B cells. Atypical cells with enlarged nuclei were present, and they were not hematopoietic but found as glial cells. JC virus (JCV) infection was verified with both immunohistochemistry and in situ hybridization; the final diagnosis was progressive multifocal leukoencephalopathy (PML). The patient was treated with mefloquine and discharged. This case is informative in understanding the host anti\u2010viral response. Variable inflammatory cells were observed, including CD4<jats:sup>+<\/jats:sup> and CD8<jats:sup>+<\/jats:sup> T cells, plasma cells, and a small amount of perivascular CD20<jats:sup>+<\/jats:sup> B cells. PD\u20101 and PD\u2010L1 expression was observed in lymphoid cells and macrophages, respectively. PML with inflammatory reactions was thought fatal, and autopsy cases of PML with immune reconstitution inflammatory syndrome (IRIS) demonstrated excessive infiltration of only CD8<jats:sup>+<\/jats:sup> T cells. However, this case revealed infiltration of variable inflammatory cells, and a favorable prognosis would be expected under PD\u20101\/PD\u2010L1 immune\u2010checkpoint regulation.<\/jats:p>","DOI":"10.1111\/neup.12929","type":"journal-article","created":{"date-parts":[[2023,7,10]],"date-time":"2023-07-10T06:47:09Z","timestamp":1688971629000},"page":"47-58","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Pathology for severe inflammatory PML with PD1\/PD\u2010L1 expression of favorable prognosis: What's a prognostic factor for PML\u2010IRIS?"],"prefix":"10.1111","volume":"44","author":[{"given":"Yukiko","family":"Shishido\u2010Hara","sequence":"first","affiliation":[{"name":"Department of Anatomic Pathology Tokyo Medical University  Tokyo Japan"},{"name":"Department of Pathology and Applied Neurobiology Kyoto Prefectural University of Medicine  Kyoto 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