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Progressive multifocal leukoencephalopathy in transplant recipients.
David Shitrit1, Nirit Lev, Ariella Bar-Gil-Shitrit
1Pulmonary Institute, Rabin Medical Center, Beilinson Campus, 49100, Petah Tiqwa, Israel. davariel@zahav.net.il
Summary
Progressive multifocal leukoencephalopathy (PML) is a rare JC virus infection in transplant recipients. This review highlights its late onset, common neurological symptoms, and typically fatal outcomes, emphasizing the need for clinical consideration.
Area of Science:
- Neurology
- Infectious Diseases
- Transplantation Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the JC virus.
- PML is an emerging opportunistic infection in organ and bone marrow transplant recipients.
- The clinical characteristics and outcomes of PML in transplant populations are not well-defined.
Purpose of the Study:
- To review and define the characteristics of PML in transplant recipients.
- To analyze the incidence, timing, clinical presentation, and outcomes of PML across different transplant types.
- To inform clinical suspicion and management strategies for PML in this vulnerable patient group.
Main Methods:
- A literature review of 24 reported cases of PML in transplant recipients.
- Analysis of transplant type, time to PML onset, presenting neurological symptoms, and treatment modalities.
- Evaluation of patient outcomes, including mortality and reported regressions.
Main Results:
- PML occurred across various transplant types, with a median onset of 17 months post-transplant (71% within 24 months).
- Kidney transplant recipients showed a trend towards later PML onset (P=0.04).
- Common symptoms included hemiparesis (50%), apathy (46%), and confusion (38%); 71% of patients died within 2.5 months.
Conclusions:
- PML is a critical neurological complication to consider in transplant recipients presenting with neurological deficits.
- While often fatal, PML management may involve immunosuppression reduction and antiviral therapy (e.g., cidofovir).
- Further research is needed to better understand and manage PML in the context of transplantation.