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mAbs directed against immunologic molecules have emerged as a new class of drugs for treating patients with various immunologic conditions. However, mAb-based treatments may confer a predisposition to various infections. The authors argue that infections in individuals treated with mAbs directed against molecules of the immune system may display some similarities to those in patients with primary immunodeficiency of the corresponding mAb target. A comprehensive dissection of the tremendously diverse human primary immunodeficiencies and the careful description of their clinical features in different populations living in diverse environments thus represents an original, neglected, but promising approach to assessing the potential risk of infection associated with therapeutic mAbs, or with any therapeutic compound inhibiting a specific immunologic molecule. [Copyright &y& Elsevier]

Primary immunodeficiency diseases (PIDs) are a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins, natural killer cells, and T and B lymphocytes. The study of these diseases has provided essential insights into the functioning of the immune system. More than 120 distinct genes have been identified, whose abnormalities account for more than 150 different forms of PID. The complexity of the genetic, immunologic, and clinical features of PID has prompted the need for their classification, with the ultimate goal of facilitating diagnosis and treatment. To serve this goal, an international committee of experts has met every 2 years since 1970. In its last meeting in Jackson Hole, Wyo, after 3 days of intense scientific presentations and discussions, the committee has updated the classification of PID, as reported in this article. [Copyright &y& Elsevier]