4.2 Article

The Effect of Intravenous Methylprednisolone on Recurrent Exacerbation in Hematologic Malignancy-associated Progressive Multifocal Leukoencephalopathy

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INTERNAL MEDICINE
卷 60, 期 8, 页码 1287-1291

出版社

JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.5917-20

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PML; hematologic malignancy-associated PML; IRIS; inflammatory PML; PML with controlled inflammatory response

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A 65-year-old man with PML after remission from acute myeloid leukemia experienced left hemiparesis episodes, responding well to steroid pulse therapy which suggests exacerbations are excessive immune responses to the JC virus, not IRIS. Neuroimaging cannot differentiate between excessive and controlled inflammation, cautioning against steroid use in the latter.
We herein report a 65-year-old man with progressive multifocal leukoencephalopathy (PML) after 2-year remission from acute myeloid leukemia who developed recurrent episodes of left hemiparesis with gadolinium enhancement on magnetic resonance imaging. Steroid pulse therapy for each exacerbation induced clinical and radiological improvement, suggesting that exacerbations are an excessive immune response to the JC virus and distinct from immune reconstitution inflammatory syndrome (IRIS). Although glucocorticoids are recommended only for IRIS, steroid pulse therapy should be considered as a therapeutic option in cases of exacerbation of hematologic malignancy-associated PML. Importantly, neuroimaging is not sufficient to differentiate excessive inflammation from a controlled inflammatory response, for which steroids are not recommended.

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