Fischer A, Griscelli C
Groupe de recherches d'immunologie et de rhumatologie pédiatriques, Hôpital des Enfants-Malades, Paris, France.
Bull Cancer. 1988;75(1):69-73.
Primary immunodeficiencies (ID) lead to a high incidence of cancer, particularly of lymphomas. The risk excess varies according to the type of ID. Cellular IDs carry a very high risk, tumoral IDs have an intermediate risk while deficiencies of non-specific immunity bear no risk. Acquired IDs are also associated with an increased risk of lymphomas. The physiopathogeny of ID-associated lymphomas remains unknown, although several hypotheses have been proposed, including a defective immunological surveillance, repeated viral and antigenic stimulations and DNA repair anomalies.