An atypical case of X-linked lymphoproliferative disease revealed as a late cerebral lymphoma.

X-linked lymphoproliferative disease (XLP) is an inherited immunodeficiency, partially characterized by a defect in cytotoxicity to Epstein-Barr virus. This viral infection is therefore often fatal in affected boys, whilst a variety of immune disorders or proliferative diseases may occur in surviving patients. We report an atypical case of a 41year-old male who presented with a primitive B-cell cerebral lymphoma, revealing an XLP. This presentation was unusual because of its late onset, the broad spectrum of the familial characteristics, its initial presentation as a cerebral lymphoma, and the occurrence of B-cell alymphocytosis associated with a-gamma-globulinemia. Copyright 2009 Elsevier B.V. All rights reserved.

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B Hervier, S Latour, D Loussouarn, M Rimbert, G De-Saint-Basile, C Picard, M Hamidou. An atypical case of X-linked lymphoproliferative disease revealed as a late cerebral lymphoma. Journal of neuroimmunology. 2010 Jan 25;218(1-2):125-8

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PMID: 19906447

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