X-linked lymphoproliferative syndrome

X-linked lymphoproliferative syndrome, a rare X-linked immunodeficiency in which there is a normal response to childhood infection but infection with Epstein-Barr produces a fatal lymphoproliferative disorder. Most patients die of acute infection. Others develop hypogammaglobulinemia, B-cell lymphoma, aplastic anemia, or agranulocytosis. See also Epstein-Barr virus.