Primary Immunodeficiency: 546 Malignancies Associated to Primary Immunodeficiencies. A 40 Year Review

摘要

BackgroundCancer has been cited as the second leading cause of death after infection in children and adults with primary immunodeficiencies (PIDs). There seems to be a complex relationship between PIDs, viral infections to which are susceptible, and the development of cancer. Defective immunosurveillance most markedly in cells with strong antigenic potential that have undergone viral induction is a major factor, as support for this the most common cancer subtype is lymphoma. Some estimates suggest that more than 20% of carcinomas in patients with PID are infection induced, Epstein Barr virus being particularly well established cofactor. The risk of cancer in patients with PID is estimated between 4 to 25%, although could be higher in some subtypes of PID. The PIDs most commonly associated to cancer are Ataxia Telangiectasia, common variable immunodeficiency, Wiscott-Aldrich syndrome, severe combined immunodeficiency, and selective Iga deficiency.