Progressive multifocal leukoencephalopathy in multiple myeloma. A literature review and lessons to learn
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REVIEW ARTICLE
Progressive multifocal leukoencephalopathy in multiple myeloma. A literature review and lessons to learn Ioannis Koutsavlis 1 Received: 17 May 2020 / Accepted: 28 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection with high mortality rate usually seen in the context of immunosuppression. Although cases have been reported largely in patients with HIV/AIDS, following the use of monoclonal antibodies and occasionally in haematological malignancies, there is no review to date of patients with smouldering or treated myeloma who developed PML. Here, we conducted a literature search of PML cases in patients with multiple myeloma (MM), analyse patient and disease characteristics and describe the possible mechanisms that could lead to the development of PML. The lack of data and case reports until 2010 may indicate that PML in MM is underdiagnosed. Simultaneously, with an expanding field of new therapeutic options, patients with MM live longer, albeit continually immunosuppressed, and at risk of opportunistic infections. Emerging new treatments for PML in the horizon render the need to look out for this complication mandatory, and more case reports are needed to enrich our knowledge in this field. Keywords Progressive multifocal leukoencephalopathy . Multiple myeloma . Haematological malignancy . Risk factors . Myeloma therapy
Introduction Progressive multifocal leukoencephalopathy (PML) constitutes in many cases a fatal disease caused by the John Cunningham (JC) virus. Immunosuppression is a prerequisite for this development where the JC virus infects oligodendrocytes in the brain and results in a serious demyelinating condition [1]. The classical presentation is that of a subacute symptomatology dating back weeks or months. The clinical symptoms may be diverse depending on the site of brain involvement but except from sensorimotor abnormalities, patients may present with subtle cognitive, behavioural or speech changes. To define the diagnostic criteria for PML, a consensus statement was developed in 2013 by the American Academy of Neurology (AAN) employing clinical, imaging, pathologic and virologic evidence. MRI of the brain is the imaging of choice where the typical finding is that of * Ioannis Koutsavlis [email protected] 1
NHS Fife, Haematology, Victoria Hospital, Kirkcaldy KY2 5AH, UK
hypointense white matter lesions on T1-weighted images and hyperintense on T2-weighted images and/or FLAIR (fluid-attenuated inversion recovery) sequences. The lesions are non-enhancing and are usually subcortical. Detection of JC virus in the cerebrospinal fluid (CSF) or brain biopsy should also be present for the diagnosis of PML. A definite diagnosis entails compatible clinical features, imaging findings and positive CSF PCR for JC virus. When a brain biopsy is performed, definitive diagnosis of PML requires neuropathologic demonstration of the typical histopathologic triad (demyelination,
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