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Epstein-Barr Virus Infections in Males with the X-Linked Lymphoproliterative Syndrome

[+] Article, Author, and Disclosure Information

Grant support: in part by Public Health Service grant CA30196 from the National Cancer Institute, Department of Health and Human Services, and by a grant from the Lymphoproliferative Research Fund.

▸Requests for reprints should be addressed to David T. Purtilo, M.D.; Department of Pathology and Microbiology, University of Nebraska Medical Center, 42nd St. & Dewey Ave.; Omaha, NE 68105-1065.

Omaha, Nebraska

© 1987 American College of PhysiciansAmerican College of Physicians

Ann Intern Med. 1987;106(4):538-545. doi:10.7326/0003-4819-106-4-538
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A registry of persons with the X-linked lymphoproliferative syndrome, which is characterized by marked susceptibility to diseases induced by the Epstein-Barr virus, has enrolled 161 patients within 44 kindreds. Fifty-seven percent of the males died of infectious mononucleosis, 29% developed acquired hypogammaglobulinemia, and 24% had malignant lymphoma. The mortality rate was 80%; 70% died by 10 years of age and 100% by 40 years. Thirty-two boys survive, most with malignant lymphoma, acquired hypogammaglobulinemia, or both. We hypothesized that the defective lymphoproliferative control locus on the X chromosome results in unregulated cytotoxic lymphocytic responses to the Epstein-Barr virus; hence, severe hepatitis and virus-associated hemophagocytic syndrome occur with the infectious mononucleosis phenotype. T-cell suppression of immunoglobulin secretion by B cells is responsible for acquired hypogammaglobulinemia. A sustained polyclonal B-cell proliferation probably converts to a monoclonal B-cell malignancy as a result of molecular alterations.





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