Azathioprine/steroids

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Progressive multifocal leukoencephalopathy and pancytopenia: case report A 71-year-old man developed pancytopenia and progressive multifocal leukoencephalopathy (PML) during treatment with azathioprine and unspecified steroids for generalised myasthenia gravis. The man presented with a progressive unilateral cerebellar syndrome. He had a significant history of generalised myasthenia gravis with acetylcholine [acethylcoline]-receptor antibodies (AchR-gMG) and resected thymoma. He had been receiving symptomatic immunosuppressive therapy with azathioprine and unspecified low dose steroids [route and dosage not stated] since 2009. He had a slight tolerated pancytopenia in 2014. At the present consultation, he underwent brain MRI which showed a T1-hypointense, T2-hyperintense lesion of the left cerebellar peduncle without contrast enhancement. Biopsy of the lesion showed demyelination with JC-virus in astrocytes and oligodendrocytes. Subsequent cerebrospinal fluid (CSF) analysis for JV-virus showed 24000 copies/mL. Based on the clinical investigation, he was diagnosed with PML secondary to immunosuppressive therapy [duration of treatment to reaction onset not stated]. Therefore, the man’s therapy with azathioprine and unspecified steroids was discontinued. After the discontinuation of therapy, JC-viral copies dropped to 360 copies/mL. However, clinical deficits remained unchanged and MRI lesions progressed. He started receiving treatment with pembrolizumab, but he died of PML 3 weeks later (14 weeks after symptom onset). Hofstadt-van Oy U, et al. Progressive multifocal leukoencephalopathy of the cerebellar peduncle in a patient with Myasthenia gravis immunosuppressed with azathioprine: Case report and review of the literature. European Journal of Neurology 27 (Suppl. 1): 994, May 2020. Available from: URL: https://onlinelibrary.wiley.com/ 803498474 toc/14681331/2020/27/S1 [abstract]

0114-9954/20/1819-0001/$14.95 Adis © 2020 Springer Nature Switzerland AG. All rights reserved

Reactions 29 Aug 2020 No. 1819

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