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Progressive multifocal leukoencephalopathy in myelodysplastic syndrome involving pure red cell aplasia

Dai Chihara1, Tomoharu Takeoka, Tomoyuki Shirase

  • 1Division of Hematology and Immunology, Department of Internal Medicine, Ohtsu Red Cross Hospital, Ohtsu.

Internal Medicine (Tokyo, Japan)
|November 5, 2010
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML), a fatal brain disease, occurred in an immunocompromised patient treated with azathioprine. JC polyomavirus (JCV) reactivation was confirmed post-mortem, suggesting azathioprine as a potential trigger for PML.

Area of Science:

  • Neuroimmunology
  • Virology
  • Hematology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating CNS disease.
  • PML results from JC polyomavirus (JCV) reactivation in immunocompromised individuals.

Observation:

  • A 76-year-old woman with myelodysplastic syndrome developed PML.
  • She had received azathioprine for a pure red cell aplasia-like condition.

Findings:

  • Neurologic symptoms, MRI findings, and JCV DNA in cerebrospinal fluid confirmed PML diagnosis.
  • Autopsy confirmed PML and detected JCV DNA in the cerebrum.
  • Azathioprine treatment is suspected as a potential trigger for PML.

Implications:

  • This case highlights the risk of PML in patients treated with immunosuppressants like azathioprine.
  • Early diagnosis and awareness of iatrogenic PML are crucial for patient management.
  • Further research is needed to understand the specific mechanisms linking azathioprine to JCV reactivation.

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