Malignancies in primary immunodeficiencies

Congenital defects of the immune system are characterized not only by frequent and prolonged infections, but also by a complex dysfunction of the immune system, which appears in the form of autoimmune and allergic processes, lymphoproliferation and malignant – primarily hematological – diseases. The incidence of malignant diseases in several primary immunodeficiencies significantly exceeds the incidence observed in the normal population. B-cell non-Hodgkin lymphomas are of outstanding importance. This observation also supports the assumption that the same genetic background (mutation) as that which caused the immunodeficiency is responsible for the increased tumor incidence in primary immunodeficiencies. In addition to these intrinsic causes, external (extrinsic) factors also play a role in the formation of tumors. Therapy is a difficult problem, because in some primary immunodeficiency conditions, patients do not tolerate conventional radiation and chemotherapy treatment well, and several infectious complications can occur. In the majority of patients, an individualized treatment strategy is required, in which the role of targeted therapeutic options becomes increasingly important.