Progressive multifocal leukoencephalopathy after liver transplantation can have favorable or unfavorable outcome

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) a...

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Bibliographic Details
Published in:Transplant infectious disease 2016-08, Vol.18 (4), p.606-610
Main Authors: Dumortier, J., Guillaud, O., Bosch, A., Coppéré, B., Petiot, P., Roggerone, S., Vukusic, S., Boillot, O.
Format: Article
Language:English
Subjects:
Adrenal Cortex Hormones - administration & dosage
Adrenal Cortex Hormones - adverse effects
Adrenal Cortex Hormones - therapeutic use
Adult
Cerebral Cortex - diagnostic imaging
Fatal Outcome
Female
Graft Rejection - drug therapy
Humans
Immunocompromised Host
Immunosuppression - adverse effects
Immunosuppression - methods
Immunosuppressive Agents - administration & dosage
Immunosuppressive Agents - adverse effects
Immunosuppressive Agents - therapeutic use
JC virus
JC Virus - isolation & purification
Leukoencephalopathy, Progressive Multifocal - cerebrospinal fluid
Leukoencephalopathy, Progressive Multifocal - diagnostic imaging
Leukoencephalopathy, Progressive Multifocal - virology
liver transplantation
Liver Transplantation - adverse effects
Magnetic Resonance Imaging
Male
polyomavirus
Prognosis
progressive multifocal leukoencephalopathy
treatment
Withholding Treatment
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Summary: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.
ISSN:1398-2273
1399-3062
DOI:10.1111/tid.12554