Intrinsic and Extrinsic Causes of Malignancies in Primary Immunodeficiency Disorders

Malignancies occur with a higher incidence rate and manifest earlier in life in patients with primary immunodeficiency disorders (PID) than in the general population. However, no universal mechanism of malignancy predisposition in PID can been determined. Despite the strong support for the physiological role of tumor immunosurveillance and increasing success of strategies in immunological tumor therapy, which include checkpoint inhibition, monoclonal antibodies, and engineered T cell antigen receptors, the incidence and pattern of malignancies in PID do not reflect an increased tumor immune escape per se.

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