Abstract: TH-PO573
PML-IRIS in a Patient with Kidney Transplant
Session Information
- Trainee Case Reports - II
October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1802 Transplantation: Clinical
Authors
- Chen, Huiwen, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
- Haidar, Ghady, UPMC, Pittsburgh, Pennsylvania, United States
Introduction
Progressive multifocal leukoencephalopathy ( PML) is a degenerative disease involving the central nervous system. It is caused by the John Cunningham virus ( JCV) which commonly affects immunocompromised patients. We present the first case of PML complicated by immune reconstitution inflammatory syndrome ( IRIS) described in a kidney transplanted patient.
Case Description
A 60 year old female with a history of kidney transplant in 2009 and native kidney failure due to IgA nephropathy who presented to the clinic with partial expressive aphasia and loss of executive function. Patient was on tacrolimus ( FK) and mycophenolate (MMF) as maintenance therapy. Physical exam does not show any facial droops or motor weakness.
MRI brain finding was concerning for PML. Lumbar puncture had positive PCR for JC virus in spinal fluid. Serum JCV viral load was positive. Remaining serology and metabolic panel were normal. MMF and subsequently FK were both removed from the regimen. Patient then developed aphasia and right sided weakness. MRI showed new mass effect. Brain biopsy was consistent with PML-IRIS. Patient was started on dexamethasone with a slow prednisone taper. She exhibited a good neurological improvement two weeks into the treatment.
Discussion
PML is a severe opportunistic infection commonly affecting patients with immunosuppressed state. Manifestations of PML include motor deficit, dysarthria, dysphasia but most commonly weakness. MRI shows hyperintense lesion on T2 at the subcortical white matter. IRIS occurs due to exaggerated T cell response in patient who was previously immunocompromised. Symptom can occur one week to few months after treatment of primary disease. PML-IRIS is well documented clinical entity in human immunodeficiency virus infection, however, there is scant data and little awareness about the presentation in transplant patients. In patient with kidney transplant, post-transplant lymphoproliferative disease is also highly on the differentials as it is common complication for all solid organ recipients. The re-activation of immune system in our patient was likely due to discontinuation of immunosuppressant in fear of progression of PML. Diagnosis would be challenging without biopsy.
Take home points:
This is the first case of PML-IRIS described in patients with kidney transplant. Patient had dramatic neurological recovery after prednisone taper, but patient is at high risk for chronic allograft rejection.