title: An atypical case of X-linked lymphoproliferative disease revealed as a late cerebral lymphoma.
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.
authors.affiliation: Internal Medicine Department, CHU NANTES, Place Alexis Ricordeau, 44093 Nantes, France. bhervier@yahoo.fr
authors.forename: B
authors.initials: B
authors.lastname: Hervier
authors.forename: S
authors.initials: S
authors.lastname: Latour
authors.forename: D
authors.initials: D
authors.lastname: Loussouarn
authors.forename: M
authors.initials: M
authors.lastname: Rimbert
authors.forename: G
authors.initials: G
authors.lastname: De-Saint-Basile
authors.forename: C
authors.initials: C
authors.lastname: Picard
authors.forename: M
authors.initials: M
authors.lastname: Hamidou
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000188:drug therapy / Q000235:genetics*; D006801:Humans; D016393:Lymphoma, B-Cell / Q000188:drug therapy / Q000235:genetics*; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000503:physiopathology; D008297:Male; D010375:Pedigree
chemical_list: D000970:Antineoplastic Agents