Fatal Hodgkin and Non-Hodgkin Lymphoma Associated with Persistent Epstein-Barr Virus in Four Brothers

  1. Rosemarie Donhuijsen-Ant, MD;
  2. Hinrich Abken, MD;
  3. Georg Bornkamm, MD;
  4. Konrad Donhuijsen, MD;
  5. Hans Grosse-Wilde, MD;
  6. Dieter Neumann-Haefelin, MD;
  7. Martin Westerhausen, MD; and
  8. Helga Wiegand, MD

    Abstract

    Three brothers from one family died of Hodgkin disease and a fourth brother from a diffuse malignant non-Hodgkin lymphoma. This patient exhibited a constant deficiency of serum immunoglobulins and elevated antibody titers to Epstein-Barr viral antigens. Epstein-Barr virus DNA sequences were detected in DNA isolated from lymph node biopsies from two of the patients. Initially, no abnormalities in the numbers of B and T cells could be detected. Peripheral blood lymphocytes of the patients did not react in the mixed lymphocyte culture assay. We suggest that an immune deficiency to Epstein-Barr virus may favor the proliferation of malignant lymphocytes after Epstein-Barr viral infection. Monoclonal lymphoid B cell lines established spontaneously in vitro from a lymph node biopsy specimen and from peripheral blood specimens from two of the patients. The cells harbor Epstein-Barr viral DNA sequences in multiple genome equivalents and express Epstein-Barr nuclear antigen. The cells contain a tenfold increased level of c-fgr-related RNA transcripts compared with peripheral blood lymphocytes of healthy adults. No obvious amplifications or translocations of the c-myc, c-abl, or c-fgr gene could be detected.

    Article and Author Information

    • From St. Johannes-Hospital, Duisburg; Institute of Genetics, Bonn; Albert-Ludwigs University, Freiburg im Breisgau; and University of Essen, Essen; Federal Republic of Germany.

    • Requests for Reprints: R. Donhuijsen-Ant, MD, Department of Hematology/Oncology/Immunology, St. Johannes-Hospital, An der Abtei 7-11, 4100 Duisburg 11, Federal Republic of Germany.

    • Current Author Addresses: Drs. Donhuijsen-Ant and Westerhausen: Department of Hematology/Oncology/Immunology, St. JohannesHospital, An der Abtei 7-11, 4100 Duisburg 11, Federal Republic of Germany.

      Dr. Abken: Institute of Genetics, Römer Str. 164, 5300 Bonn 1, Federal Republic of Germany.

      Drs. Bornkamm, Neumann-Haefelin, and Wiegard: Department of Virology, Institute for Microbiology and Hygiene, Albert-Ludwigs University, Hermann-Herder Str. 11, 7800 Freiburg im Breisgau, Federal Republic of Germany.

      Dr. Donhuijsen: Institute of Pathology, University of Essen, Hufelandstr. 55, 4300 Essen 1, Federal Republic of Germany.

      Dr. Grosse-Wilde: Department of Immunogenetics, University of Essen, Virchowstrasse 171, 4300 Essen 1, Federal Republic of Germany.

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    1. Ann Intern Med December 15, 1988 vol. 109 no. 12 946-952
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