Nov 5, 2010

Progressive multifocal leukoencephalopathy in myelodysplastic syndrome involving pure red cell aplasia

Internal Medicine
Dai ChiharaTatsuharu Ohno


Progressive multifocal leukoencephalopathy (PML) is a rare and fatal demyelinating disease of the central nervous system caused by JC polyomavirus (JCV) reactivation in an immunocompromised host. We describe a case of PML in a 76-year-old woman with myelodysplastic syndrome, who had been treated with azathioprine for a pure red cell aplasia-like condition. PML was diagnosed based on the neurologic symptoms, the magnetic resonance imaging patterns and the detection of JCV DNA in the cerebrospinal fluid. She died ten months after the diagnosis. An autopsy confirmed the diagnosis, and JCV DNA was detected in the cerebrum. Azathioprine might have triggered PML.

Mentioned in this Paper

Leukoencephalopathy, Progressive Multifocal
Magnetic Resonance Imaging
Hematopoetic Myelodysplasia
Cerebrospinal Fluid
Cortical Cell Layer of the Cerebral Cortex

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