[Primary immunodeficiencies and hematological malignancies]

Primary immunodeficiency disease (PID) is characterized by an impaired immune system along with recurrent and severe and/or opportunistic infections; these infections are frequently associated with malignancies and autoimmune disorders. Dysregulation of the adaptive immune system leads to an increased risk of malignancies. Various mechanisms contribute to increased tumor susceptibility in patients with PIDs, which are as follows: defective cell-mediated tumor surveillance, defective apoptosis, specific susceptibility to carcinogens, decreased pathogen clearance and over-active inflammatory responses, defects in cytokinesis, impaired control of virus-infected cells, defects in cell cycle control, and impaired cellular response to genotoxic agents. Moreover, some malignancies might arise owing to germline mutations in PID-causative genes. Here, we describe the close association between PIDs and malignancies, particularly hematopoietic malignancies.