Primary immunodeficiencies: genetic risk factors for lymphoma.
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.
Filipovich
A H
AH
Immunodeficiency Cancer Registry, University of Minnesota, Minneapolis 55455.
Mathur
A
A
Kamat
D
D
Shapiro
R S
RS
D000293:Adolescent; D000328:Adult; D001260:Ataxia Telangiectasia / Q000150:complications / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000235:genetics* / Q000382:microbiology; D007223:Infant; D008223:Lymphoma / Q000209:etiology / Q000235:genetics* / Q000382:microbiology; D016393:Lymphoma, B-Cell / Q000209:etiology / Q000235:genetics / Q000382:microbiology; D008297:Male; D008875:Middle Aged; D012307:Risk Factors