Malignancies in the setting of primary immunodeficiency: Implications for hematologists/oncologists

Many primary immunodeficiency disorders (PIDD) are associated with elevated risks for different types of cancer. Defective immunosurveillance mechanisms in PIDD and infection with oncogenic viruses (eg, Epstein Barr, herpesvirus 8) seem to have significant contributory roles in many cases. Non-Hodgkin lymphoma and Hodgkin disease are two of the most common PIDD-associated malignancies. The impact of PIDD-associated malignancy has increased in recent years in parallel with improved patient with PIDD survival and longevity, due largely to effective immunoglobulin replacement therapy. Epidemiologic data, clinical patterns, and management considerations of the common PIDD-associated cancers are reviewed.