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Cancer Res. 1992 Oct 1;52(19 Suppl):5465s-5467s.

Primary immunodeficiencies: genetic risk factors for lymphoma.

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  • 1Immunodeficiency Cancer Registry, University of Minnesota, Minneapolis 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.

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