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Documentation of Epstein-Barr Virus Infection in Immunodeficient Patients with Life-threatening Lymphoproliferative Diseases by Clinical, Virological, and Immunopathological Studies¹

Department of Pathology [D. T. P., K. S.] and Pediatrics [D. T. P., J. L. S.], University of Massachusetts, Worcester, Massachusetts 01605; Department of Tumor Biology, Karolinska Institute, S-104 01 Stockholm, Sweden [D. T. P., A. K. S., A-C. S., M. A., G. K.]; Hospital for Sick Children, Great Ormond Street, London, England [J. P., C. S., C. S., J. P.]; Department of Pediatrics, Children's Memorial Hospital, Chicago, Illinois [L. P., K. R., F. C.]; Department of Pediatrics, Southwestern Medical School, Dallas, Texas [J. A. C.]; Department of Pathology, Vanderbilt University Medical School, Nashville, Tennessee [R. C.]; Department of Pediatrics, Addenbrooke Hospital, Cambridge, England [N. B., J. K.]; and Department of Pathology, Danderyd Hospital, Stockholm, Sweden [B. S.]

Multiple methods, pedigree analysis, clinical evaluation, and Epstein-Barr virus (EBV)-specific serology, EBV DNA hybridization of tissues to probe for viral genome, staining of touch imprints for EBV nuclear-associated antigen, establishment of spontaneous infected B-cell lines from peripheral blood or tissues, examination of peripheral blood smears, and hematopathology studies, were used to study seven patients with the X-linked lymphoproliferative syndrome and seven additional patients with life-threatening EBV-associated diseases. These studies demonstrated EBV in the tissues of all 14 patients and immunodeficient antibody responses to EBV were documented. This virus can produce various life-threatening lymphoproliferative diseases in a variety of immunodeficient patients.

¹ This work was supported in part by NIH Grant CA 23561, X-Linked Lymphoproliferative Fund, and NIH Contract NO1CP33316.

² Recipient of a Fogarty Senior International Fellowship. To whom requests for reprints should be addressed, at Department of Pathology and Laboratory Medicine, University of Nebraska Medical Center, 42nd and Dewey Avenue, Omaha, Nebr. 68105.

Received 11/20/80. Accepted 4/ 2/81.