An atypical case of X-linked lymphoproliferative disease revealed as a late cerebral lymphoma
Received 3 August 2009; received in revised form 12 October 2009; accepted 19 October 2009. published online 11 November 2009.
Abstract
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.
ABSTRACT