Journal of Clinical Immunology

, Volume 4, Issue 5, pp 369–382 | Cite as

Epstein-Barr virus (EBV)-specific cell-mediated and humoral immune responses in ataxia-telangectasia patients

  • Giuseppe Masucci
  • Izzet Berkel
  • Maria Grazia Masucci
  • Ingemar Ernberg
  • Robert Szigeti
  • Fugen Ersoy
  • Özden Sanal
  • Olcay Yegin
  • Gertrude Henle
  • Werner Henle
  • Gary Pearson
  • Pierre Åman
  • George Klein
Original Articles

Abstract

As a part of studies on cell-mediated immune (CMI) responses of immunocompromised, Epstein-Barr virus (EBV)-infected patients who can or cannot restrict the proliferation of EBV-transformed B cells, we have studied 16 Turkish patients with ataxia-telangectasia (AT). Fifteen were EBV seropositive; one was seronegative. Among the seropositives, eight had no or only low anti-EBV-determined nuclear antigen (EBNA) antibody titers, while seven had normal anti-EBNA levels. EBV-seropositive and -seronegative healthy Turkish children were used as controls. We have particularly asked the question whether low EBNA antibody titers can be correlated with the level of EBV-specific and -nonspecific cell-mediated immunity. Non-EBV-specific tests included cell count, phenotypical characterization with monoclonal antibodies, assessment of natural killer (NK)-cell activity, and ability to suppress mitogen-induced immunoglobulin production. Two EBV-specific CMI tests were used: outgrowth inhibition (OI) and leukocyte migration inhibition (LMI). The majority of the patients of the low-EBNA antibody group was IgA deficient and had high levels of α-fetoprotein (a-FP). Cells reacting with OKT8 monoclonal antibody predominated in both AT patient groups. In contrast, the suppressor activity was present in only a few patients and NK and interferon-activated killing (IAK) activities were normal. EBV-specific cell-mediated responses were defective in seven of eight patients in the low-anti-EBNA group and five of seven patients in the group with normal anti-EBNA titers. It is concluded that AT patients are often defective in their EBV-specific cell-mediated immune responses and with regard to their EBNA antibody levels. These defects are associated with a predominance of T cells reacting with OKT8 monoclonal antibody.

Key words

Ataxia-Telangectasia Epstein-Barr virus immunodeficiency 

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Copyright information

© Plenum Publishing Corporation 1984

Authors and Affiliations

  • Giuseppe Masucci
    • 1
  • Izzet Berkel
    • 2
  • Maria Grazia Masucci
    • 1
  • Ingemar Ernberg
    • 1
  • Robert Szigeti
    • 1
  • Fugen Ersoy
    • 2
  • Özden Sanal
    • 2
  • Olcay Yegin
    • 2
  • Gertrude Henle
    • 3
    • 4
  • Werner Henle
    • 3
    • 4
  • Gary Pearson
    • 5
  • Pierre Åman
    • 1
  • George Klein
    • 1
  1. 1.Department of Tumor BiologyKarolinska InstitutetStockholmSweden
  2. 2.Institute of Child HealthHacettepe University, HacettepeAnkaraTurkey
  3. 3.The Joseph Stokes, Jr., Research InstituteThe Children Hospital of PhiladelphraPhiladelphia
  4. 4.School of MedicineUniversity of PennsylvaniaPhiladelphia
  5. 5.Mayo ClinicSection of Microbiology Rochester

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