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

Insights

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.

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