Primary Immunodeficiencies and Cancers Publisher



Hedayat M¹ ; Alherz W² ; Aghamohammadi A³ ; Nichols KE⁴ ; Rezaei N^{5, 6}
Source: Cancer Immunology: A Translational Medicine Context Published:2015

Abstract

Primary immunodeficiencies (PIDs) comprise a heterogeneous group of genetic disorders, predisposing to frequent and severe infections, autoimmunity, and cancer. In recent years, the tendency to develop cancers has increased in parallel with improved life expectancy, largely due to effective immunoglobulin replacement and other supportive therapies. The overall risk for developing malignancies in children with PIDs is 4a€”25%, with lymphomas representing up to 60% of cancers. Defective immunosurveillance mechanisms and infection with oncogenic viruses have significant contributory roles in many cases. In this chapter, we review our understanding of the spectrum and pathogenesis of malignancies in the context of immunodeficiency syndromes, with emphasis on the epidemiologic data, clinical patterns, and potential importance of cancer screening for patients with specific PIDs. © Springer-Verlag Berlin Heidelberg 2015.