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

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.

A case of progressive multifocal leukoencephalopathy in a post-kidney transplant patient with improvement after discontinuation of immunosuppressive drugs and combination therapy with mefloquine and mirtazapine

“…In cases of drug-related PML caused by the use of biological agents or immunosuppressive drugs, reducing or discontinuing the drug and considering plasma exchange are recommended. Non-HIV-PML patients often have favorable outcomes when immunosuppression is reduced [11]. In this case, our patient had a positive outcome, which we attribute to the discontinuation of immunosuppressive drugs.…”

Progressive multifocal leukoencephalopathy presenting with acute sensorineural hearing loss in an intestinal transplant recipient

“…The mainstay of treatment is reducing immunosuppression, with a focus on restoring T‐cell function. Case reports of HIV‐negative patients with PML associate reducing immunosuppression with favorable outcomes 35 . For our patient, reduction and ultimately discontinuation of immunosuppression to restore immune function were attempted as a life‐saving measure despite the resulting increased risk of organ rejection.…”

“…34 Therefore, we are unable to Case reports of HIV-negative patients with PML associate reducing immunosuppression with favorable outcomes. 35 For our patient, reduction and ultimately discontinuation of immunosuppression to restore immune function were attempted as a life-saving measure despite the resulting increased risk of organ rejection. JC viral loads decreased after this intervention, though additional antiviral agents discussed below were added at the same time.…”