Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection

Motohiko Okano, Geoffrey M. Thiele, Roger H. Kobayashi, Jack R. Davis, Mark S. Synovec, Helen L. Grierson, Howard S. Jaffe, David T. Purtilo

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32 Scopus citations


A 20-month-old male with fulminant infectious mononucleosis and the X-linked lymphoproliferative syndrome (XLP) was studied. Epstein-Barr virus (EBV)-determined nuclear antigen (EBNA) and EBV DNA were detected in various tissues. Despite a combined treatment with acyclovir, immunoglobulin, and methylprednisolone, the patient deteriorated rapidly. Following treatment with recombinant interferon-gamma (IFN-γ), defervescence occurred and circulating EBNA-positive cells markedly decreased. IFN-γ prior to treatment ranged from 10.8 to 24.5 U/ml in the patient's serum and increased linearly post exogenous IFN-γ treatment. His natural killer (NK)-cell activity remained in the normal range throughout his illness but autologous EBV-infected cells were not killed in vitro by his peripheral blood lymphocytes (PBL). These results suggest that patients with the fatal infectious mononucleosis phenotype of XLP may produce endogenous IFN-γ. Defective cytotoxic T cells against EBV-infected cells seem to be responsible for the fulminant infectious mononucleosis in this patient.

Original languageEnglish (US)
Pages (from-to)48-54
Number of pages7
JournalJournal of Clinical Immunology
Issue number1
StatePublished - Jan 1989


  • Epstein-Barr virus
  • X-Linked lymphoproliferative syndrome
  • cytotoxic T cell
  • interferon-gamma
  • natural killer cell

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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