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Primary Immunodeficiencies: Genetic Risk Factors for Lymphoma¹

Immunodeficiency Cancer Registry [A. H. F.] and Division of Immunology, Department of Pediatrics, [A. H. F., A. M., D. K., R. S. S.], and the Department of Oral Sciences [A. M.], University of Minnesota, Minneapolis, Minnesota 55455

It has been estimated that up to 25% of patients with certain genetically determined immunodeficiencies will develop tumors, primarily B-cell lymphomas, during their lifetime. Epstein-Barr virus appears to be an important cofactor in the development of lymphoproliferative disorders in patients with primary immunodeficiencies, as well as acquired immunodeficiencies. Additionally, host defects in immunoregulation and/or gene rearrangement, which are features of certain primary immunodeficiencies, probably contribute to the risk of lymphomagenesis in patients at risk.