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Published on: November 7, 2020
Progressive multifocal leukoencephalopathy after liver transplantation can have favorable or unfavorable outcome
J Dumortier1,2, O Guillaud1, A Bosch1,2
1Fédération des Spécialités Digestives, Hospices civils de Lyon, Hôpital Edouard Herriot, Lyon, France.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the JC polyomavirus (JCPyV) in immunocompromised patients, including solid organ transplant recipients. We report 2 cases of PML late after liver transplantation (144 and 204 months) and review the few other published cases. The clinical course of PML is characterized by a rapid progressive neurological decline coinciding with the presence of white matter lesions on magnetic resonance images. No direct antiviral therapy is available against the JCPyV. The prognosis is therefore extremely poor. Restoration of the immune response achieved by tapering or ending the immunosuppressive therapy is the basis of treatment in transplanted patients. One of our patients is alive 3 years after diagnosis after total withdrawal of immunosuppressive therapy. The other presented severe rejection when tapering immunosuppression and died 26 months after diagnosis.
Insights
Progressive multifocal leukoencephalopathy (PML) is a rare brain infection in immunocompromised patients. Managing PML in liver transplant recipients involves carefully restoring immune function, with outcomes varying based on immunosuppression withdrawal.
Area of Science:
- Neuroscience
- Virology
- Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a serious demyelinating disease affecting the central nervous system.
- PML is caused by the JC polyomavirus (JCPyV), primarily in immunocompromised individuals, including solid organ transplant recipients.
Observation:
- This report details two cases of late-onset PML occurring 144 and 204 months post-liver transplantation.
- The clinical presentation of PML involves rapid neurological decline and characteristic white matter lesions on MRI.
Findings:
- There is no specific antiviral treatment for JCPyV, leading to a poor prognosis.
- Restoring the immune response by reducing immunosuppression is the cornerstone of treatment for transplant patients.
- One patient survived 3 years post-diagnosis after complete immunosuppression withdrawal, while another died 26 months post-diagnosis due to severe rejection during immunosuppression tapering.
Implications:
- These findings highlight the critical role of immune reconstitution in managing PML in liver transplant recipients.
- Careful management of immunosuppression is essential to balance the risk of PML with the prevention of organ rejection.
- Further research into JCPyV pathogenesis and treatment strategies is warranted for this vulnerable patient population.
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