The X-Linked Lymphoproliferative Disease: From Autopsy Toward Cloning the Gene 1975–1990
- 1 January 1991
- journal article
- review article
- Published by Taylor & Francis in Pediatric Pathology
- Vol. 11 (5) , 685-710
- https://doi.org/10.3109/15513819109065466
Abstract
Although X-linked lymphoproliferative disease (XLP) is rare (1–2 males per 1 × 106), it serves as a model for discerning diverse diseases caused by Epstein-Barr virus (EBV) ranging from agammaglobulinemia to fatal infectious mononucleosis following infection with the virus. The study of patients with XLP has also paved the way to understanding how EBV induces diseases in children with primary immunodeficiency diseases, organ transplant recipients, and those with acquired immunodeficiency syndrome. This review is dedicated to the memory of Gordon Vawter, M.D., who generously provided insights into the causes of pathogenesis of immune deficiency and lymphoproliferative disorders.Keywords
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