X-linked lymphoproliferative syndrome registry report. NLM AIDSLINE Important note: Information in this article was accurate in 1980. The state of the art may have changed since the publication date.

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X-linked lymphoproliferative syndrome registry report.

J Pediatr. 1980 Apr;96(4):669-73. Unique Identifier : AIDSLINE MED/80139593
Hamilton JK; Paquin LA; Sullivan JL; Maurer HS; Cruzi FG; Provisor AJ; Steuber CP; Hawkins E; Yawn D; Cornet JA; Clausen K; Finkelstein GZ; Landing B; Grunnet M; Purtilo DT

Abstract: Immune deficiency, especially to the Epstein-Barr virus, and increased susceptibility to fatal infectious mononucleosis, acquired agammoglobulinemia, and lymphoma are the cardinal features of the X-linked lymphoproliferative syndrome. Since the establishment of the XLP Registry in September, 1978, 59 affected males in seven unrelated kindreds were comprehensively studied. A spectrum of lymphoproliferative phenotypes was observed. Thirty-four patients (57%) died from infectious mononucleosis, eight (14%) had fatal infectious mononucleosis with lymphoma (immunoblastic sarcoma), nine (15%) had depressed immunity following EBV infection, and eight (14%) developed lymphoma. Several patients with XLP lacked EBV antibodies despite infection by EBV. The results of this study suggest that EBV can be an oncogenic agent in patients who are immune deficient with XLP.
Keywords: Adolescence Adult Burkitt's Lymphoma/GENETICS Child Child, Preschool Female Human Infant Infectious Mononucleosis/GENETICS Linkage (Genetics) *Lymphoproliferative Disorders/GENETICS Male *Registries Support, U.S. Gov't, P.H.S. Syndrome X Chromosome JOURNAL ARTICLEKWDadolescenceadultburkitt'slymphoma/geneticschildchild,preschoolfemalehumaninfantinfectiousmononucleosis/geneticslinkage(genetics)KWDlymphoproliferativedisorders/geneticsmaleKWDregistriessupport,uKWDsKWDgov't,pKWDhKWDsKWDsyndromexchromosomejournalarticle
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Copyright © 1980 - National Library of Medicine. Reproduced under license with the National Library of Medicine, Bethesda, MD.

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