Journal of NeuroVirology

, Volume 23, Issue 4, pp 632–636 | Cite as

Progressive multifocal leukoencephalopathy in a patient with lymphoma and presumptive hyper IgE syndrome

  • Rahsan Gocmen
  • Nazire Pinar AcarEmail author
  • Deniz Cagdas
  • Asli Kurne
Case Report


We, herein, report a 23-year-old male with a rare inherited immunodeficiency disease, hyperimmunoglobulin IgE syndrome (HIES), who developed progressive multifocal leukoencephalopathy (PML) and lymphoma simultaneously. Primary immunodeficiency of the patient has remained undiagnosed until adulthood. PML is a severe demyelinating disease of the central nervous system caused by John Cunningham virus. HIES is a rare, inherited immunodeficiency characterized by high serum levels of IgE, recurrent staphylococcal infection, eczema, and hypereosinophilia. PML may accompany primary immunodeficiency syndromes, but the association with HIES is exceedingly rare. We discuss the imaging findings, medical management, and a review of related literature on primary immunodeficiency cases complicating with PML.


Progressive multifocal leukoencephalopathy (PML) Hyperimmunoglobulin E syndrome (HIES) B cell lymphoma JC virus 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Journal of NeuroVirology, Inc. 2017

Authors and Affiliations

  1. 1.Department of Radiology, Faculty of MedicineHacettepe UniversityAnkaraTurkey
  2. 2.Department of Neurology, Faculty of MedicineHacettepe UniversityAnkaraTurkey
  3. 3.Department of Pediatric Immunology, Faculty of MedicineHacettepe UniversityAnkaraTurkey

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