Lymphoma complicating primary immunodeficiency syndromes

Abstract

One of the strongest arguments for the immune surveillance network of antibodies and sensitized cytotoxic T cells is the extraordinary incidence of lymphoid malignancy in the many types of primary immunodeficiency. This review updates the literature that seems to wax and wane on the importance of specific immunity to malignant cell antigens by previous authors. This new survey strongly supports the tenet that immune responses protect from malignant cell growth and development, much like it protects against a hostile microbial world. As more genetic lesions are discovered that cause various forms of immune deficiency, each with their consequent type of infection or malignancy, the notion that certain devastating infections or malignancies develop by chance is becoming less likely. The predominance of B-cell lymphoma in immune-deficient patients is both interesting and vexing. One might reasonably ask why the entire spectrum of malignancies is not seen in primary immunodeficiency. Regardless of continuing debate on immune surveillance of malignancy, the evidence presented in this review strongly supports that it has a key role in preventing lymphoid malignancy.