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Published in: Journal of NeuroVirology 4/2017

01-08-2017 | Case Report

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

Authors: Rahsan Gocmen, Nazire Pinar Acar, Deniz Cagdas, Asli Kurne

Published in: Journal of NeuroVirology | Issue 4/2017

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Abstract

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.
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Metadata
Title
Progressive multifocal leukoencephalopathy in a patient with lymphoma and presumptive hyper IgE syndrome
Authors
Rahsan Gocmen
Nazire Pinar Acar
Deniz Cagdas
Asli Kurne
Publication date
01-08-2017
Publisher
Springer International Publishing
Published in
Journal of NeuroVirology / Issue 4/2017
Print ISSN: 1355-0284
Electronic ISSN: 1538-2443
DOI
https://doi.org/10.1007/s13365-017-0536-x

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