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Published on: October 27, 2014
Sequential treatment of progressive multifocal leukoencephalopathy with intravenous immunoglobulins and pembrolizumab
Fabian Boesl1, Kristina Allers2, Juliane Herm3
1Department of Neurology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt Universität Zu Berlin, 12200, Berlin, Germany. fabian.boesl@charite.de.
Insights
Progressive multifocal leukoencephalopathy (PML), a rare CNS disease, may benefit from a combination therapy. This approach included intravenous immunoglobulins, maraviroc, and pembrolizumab, showing positive outcomes in one patient.
Area of Science:
- Neuroimmunology
- Viral Diseases of the Central Nervous System
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, severe demyelinating disease of the central nervous system (CNS).
- PML is caused by the human polyomavirus 2 (JCV) and primarily affects immunocompromised individuals.
- Currently, no specific antiviral therapy exists for PML; immune system restoration is the main treatment strategy.
Observation:
- A case series in 2019 suggested pembrolizumab, a PD-1 inhibitor, as a potential PML treatment.
- Subsequent case reports have not consistently confirmed positive outcomes with pembrolizumab monotherapy.
- Pembrolizumab targets the PD-1 receptor on lymphocytes, potentially enhancing virus-specific T cell responses.
Findings:
- This report details a patient diagnosed with PML.
- The patient received a combination treatment regimen.
- The regimen included intravenous immunoglobulins (IVIg), maraviroc, and pembrolizumab.
Implications:
- This combined treatment approach demonstrated a beneficial outcome in a PML patient.
- Further investigation into combination therapies for PML is warranted.
- This case highlights a potential therapeutic avenue for managing PML in immunocompromised patients.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the CNS caused by the human polyomavirus 2 (JCV). PML predominantly occurs in immunocompromised patients. To date, no specific antiviral treatment exists, leaving only restoration of the immune system as possible treatment. In 2019, the monoclonal antibody pembrolizumab was reported as a potential treatment option in PML in a case series. Following case reports could not thoroughly confirm a positive outcome. Pembrolizumab targets the inhibitory programmed cell death protein 1 (PD-1) receptor on lymphocytes and is associated with beneficial expansion of pre-existing virus-specific T cells. Here we describe a patient with PML who benefited from combined treatment with intravenous immunoglobulins, maraviroc, and pembrolizumab.
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