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

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

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

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
Volume9
Issue number1
DOIs
StatePublished - Jan 1 1989

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Epstein-Barr Virus Infections
Lymphoproliferative Disorders
Infectious Mononucleosis
Interferon-gamma
Human Herpesvirus 4
Epstein-Barr Virus Nuclear Antigens
Acyclovir
Methylprednisolone
Therapeutics
Natural Killer Cells
Immunoglobulins
Reference Values
Lymphocytes
T-Lymphocytes
Phenotype
DNA
Serum

Keywords

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

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection. / Okano, Motohiko; Thiele, Geoffrey Milton; Kobayashi, Roger H.; Davis, Jack R.; Synovec, Mark S.; Grierson, Helen L.; Jaffe, Howard S.; Purtilo, David T.

In: Journal of Clinical Immunology, Vol. 9, No. 1, 01.01.1989, p. 48-54.

Research output: Contribution to journalArticle

Okano, M, Thiele, GM, Kobayashi, RH, Davis, JR, Synovec, MS, Grierson, HL, Jaffe, HS & Purtilo, DT 1989, 'Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection', Journal of Clinical Immunology, vol. 9, no. 1, pp. 48-54. https://doi.org/10.1007/BF00917127
Okano, Motohiko ; Thiele, Geoffrey Milton ; Kobayashi, Roger H. ; Davis, Jack R. ; Synovec, Mark S. ; Grierson, Helen L. ; Jaffe, Howard S. ; Purtilo, David T. / Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection. In: Journal of Clinical Immunology. 1989 ; Vol. 9, No. 1. pp. 48-54.
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