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Volume 297:1077-1080 November 17, 1977 Number 20 Next

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Abstract

Investigation of a family with cancer in boys revealed that at least 20 males had the X-linked recessive lymphoproliferative syndrome. A variety of phenotypes occurred: aproliferative phenotypes consisted of aplastic anemia, agranulocytosis or acquired hypogammaglobulinemia; and proliferative phenotypes of B cells included disorders associated with the Epstein-Barr virus, American Burkitt's lymphoma, immunoblastic sarcoma of B cells, fatal infectious mononucleosis or plasmacytoma. The lymphoproliferative disorders observed in males could have resulted from an immunodeficiency to Epstein-Barr virus. The variable phenotypic expression could have resulted from individual differences in the viral dose, duration of exposure and age at which the boys were exposed to the virus. Aproliferative phenotypes such as acquired hypogammaglobulinemia could have ensued from excessive suppressor-cell activity on B cells, whereas proliferative phenotypes such as Burkitt's lymphoma or fatal infectious mononucleosis could have resulted from infection by Epstein-Barr virus and failure to stop proliferation of B cells.

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• Cho, Y.-G., Ramer, J., Rivailler, P., Quink, C., Garber, R. L., Beier, D. R., Wang, F. (2001). An Epstein-Barr-related herpesvirus from marmoset lymphomas. Proc. Natl. Acad. Sci. USA 98: 1224-1229 [Abstract] [Full Text]  
• Arkwright, P. D, Makin, G., Will, A. M, Ayres, M., Gokhale, D. A, Fergusson, W. D, Taylor, G M. (1998). X linked lymphoproliferative disease in a United Kingdom family. Arch. Dis. Child. 79: 52-55 [Abstract] [Full Text]  
• Porta, G, MacMillan, S, Nagaraja, R, Mumm, S, Zucchi, I, Pilia, G, Maio, S, Featherstone, T, Schlessinger, D (1997). 4.5-Mb YAC STS contig at 50-kb resolution, spanning Xq25 deletions in two patients with lymphoproliferative syndrome.. Genome Res 7: 27-36 [Abstract]