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

• Published in: Transplant infectious disease Vol. 18; no. 4; pp. 606 - 610
• Main Authors: Dumortier, J., Guillaud, O., Bosch, A., Coppéré, B., Petiot, P., Roggerone, S., Vukusic, S., Boillot, O.
• Format: Journal Article
• Language: English
• Published: Denmark Wiley Subscription Services, Inc 01.08.2016
• 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; polyomavirus; treatment; JC virus; progressive multifocal leukoencephalopathy; prognosis; liver transplantation
• ISSN: 1398-2273; 1399-3062
• DOI: 10.1111/tid.12554

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.