Occurrence of malignancy in immunodeficiency diseases: A literature review

The incidence of malignancy in patients with primary immunodeficiencies is roughly 10,000 times that of the general age-matched population. It is apparent from this review of the literature that each type of immunodeficiency has a distinctive constellation of malignancies associated with it. In light of recent studies demonstrating both immunologically aggressive lymphocytes and the presence of blocking antibodies in the blood of neuroblastoma patients, a major role for immunity in ontogenesis seems almost certain. The role of such antibodies in the formation of lymphoid malignancies, such as occur so frequently in patients with immunologic deficiencies who often do not produce antibodies of any type well, remains unclear.