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.
Hervier
B
B
Internal Medicine Department, CHU NANTES, Place Alexis Ricordeau, 44093 Nantes, France. bhervier@yahoo.fr
Latour
S
S
Loussouarn
D
D
Rimbert
M
M
De-Saint-Basile
G
G
Picard
C
C
Hamidou
M
M
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
D000970:Antineoplastic Agents