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Diverse Familial Malignant Tumors and Epstein-Barr Virus¹

Departments of Pathology [D. T. P., S. A. L., K. S., D. F., L. P., K. M.] and Laboratory Medicine [P. R.], University of Massachusetts Medical Center, Worcester, Massachusetts 01605; Department of Pathology [J. P. S. Y.] and Medicine [L. M. S.] of St. Vincent Hospital, Worcester, Massachusetts 01605; Department of Bacteriology and Immunology and Cancer Research Center, University of North Carolina, Chapel Hill, North Carolina 27514 [L. M. H-F.]; and Tumor Biology, Karolinska Institute, Stockholm, Sweden [A. K. S., G. K.]

Continued monitoring of a family for new malignant tumors has revealed diverse immunological and neoplastic disorders during a 15-year period. In 1966, the proband developed lymphoma. In 1975, his antibody titers to Epstein-Barr virus (EBV) became elevated, and again, he developed a malignant lymphoma. He also had borderline hypo-immunoglobulin A, died of glioblastoma multiforme in 1977, and at autopsy, had adenomatous colonic polyps. His eldest brother has normal immunoglobulin levels, but developed immune thrombocytopenia in 1973 and had elevated EBV antibody titers in 1980. Another brother had hypo-immunoglobulin A, thymoma in 1965, and adenomas and adenocarcinoma of the colon. Two other brothers succumbed to glioblastoma in 1968 and 1969. The father of the proband had bronchiectasis in 1952, hypo-immunoglobulin M documented in 1972, and elevated EBV antibody titers 5 years preceding development of a malignant lymphoma. The latter contained 10 EBV genome equivalents/cell by EBV viral DNA/DNA reassociation kinetics analysis. The proband's grandmother had died of an immunoglobulin G-secreting myeloma in 1977, and his grandfather had borderline low immunoglobulin M, elevated EBV antibody titers, and hypopharyngeal carcinoma in 1980. Predisposition to oncogenesis in this family was probably inherited.

¹ Supported in part by American Cancer Society Grant RD-15, NIH Grant CA2356¹, NIH Contract NOICP33316, the Virus Cancer Program, and the Swedish Cancer Society.

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

Received 11/20/80. Accepted 6/23/81.