EBV driven natural killer cell disease of the central nervous system presenting as subacute cognitive decline

Publication date: November 2017 Source:Human Pathology: Case Reports, Volume 10 Author(s): Francesca M. Brett, Richard Flavin, Daphne Chen, Teresa Loftus, Seamus Looby, Allan McCarthy, Cillian de Gascun, Elaine S. Jaffe, Nurul Nor, Mohsen Javadpour, Dominick McCabe Brain biopsy in patients presenting with subacute encephalopathy is never straightforward, and only undertaken when a ‘treatable condition’ is a realistic possibility. This 63year old right handed, immunocompetent Caucasian woman presented with an 8month history of rapidly progressive right-sided hearing impairment, a 4month history of intermittent headaches, tinnitus, ‘dizziness’, dysphagia, nausea and vomiting, with the subsequent evolution of progressive gait ataxia and a subacute global encephalopathy. The possibility of CJD was raised. Brain biopsy was carried out. Western blot for prion protein was negative. She died 9days later and autopsy brain examination confirmed widespread subacute infarction due to an EBV positive atypical NK/T-cell infiltrate with positivity for CD3, CD56, granzyme B, perforin and EBER with absence of CD4, CD5 and CD8 expression. Molecular studies for T-cell clonality were attempted but failed due to insufficient DNA quality. Serology was consistent with past EBV infection (EBV VCA and EBNA IgG Positive). There was no evidence of disease outside the CNS. Primary central nervous system NK/T-cell lymphoma is extremely rare. The rare reported cases all present with a...
Source: Human Pathology: Case Reports - Category: Pathology Source Type: research