Abstract: FR-PO747
Progressive Multifocal Leukoencephalopathy (PML): A Dreaded Complication of Immunosuppression
Session Information
- Post-Transplantation and Case Reports
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2102 Transplantation: Clinical
Authors
- Hernandez Garcilazo, Nora H., Yale School of Medicine, New Haven, Connecticut, United States
- Virmani, Sarthak, Yale School of Medicine, New Haven, Connecticut, United States
- Asch, William S., Yale School of Medicine, New Haven, Connecticut, United States
Introduction
JC virus (JCV) can cause progressive multifocal leukoencephalopathy (PML), a demyelinating disorder, in immunosuppressed patients. This infection may present with a variety of neurologic symptoms, ranging in severity and duration, usually identified by the area of white matter lesion. We share a case of a patient who presented with neurological weakness & was found to have PML in the cerebellar region yet no signs of cerebellar dysfunction.
Case Description
A 77-year-old lady, PMH of ESKD due to RPGN received a live donor KT in 2021, on MMF, prednisone & belatacept, presented with lower extremity motor weakness for 2 months without sensory deficits. No cause was identified on clinical exam & lab work. Allograft function was intact. A brain MRI showed a left cerebellar peduncle lesion and surrounding enhancement which did not explain her weakness.
This prompted a biopsy that showed features of PML. CSF later resulted positive for JCV by PCR.
To reduce her immunosuppression(IS) and belatacept effect, a plasmapheresis session was performed followed by administration of one dose of IVIG 25g.
Unfortunately, the next day she had worsening headaches prompting a CT which revealed a large bleed in posterior fossa originating from the biopsy site. Given poor neurological prognosis, decision was made to transition to hospice care, and she died shortly after.
Discussion
PML is catastrophic in patients on chronic IS. It's insidious presentation makes it challenging for an accurate & timely diagnosis. While the site of white matter involvement is associated with signs & symptoms, one must be mindful that lack of classical findings should not rule out a diagnosis of PML. Lack of targeted treatment for JCV infection leaves (IS) withdrawal as the only option which becomes challenging in patients on novel agents with more prolonged half-lives.