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Primary Immunodeficiencies and Cancers Publisher



Hedayat M1 ; Alherz W2 ; Aghamohammadi A3 ; Nichols KE4 ; Rezaei N5, 6
Authors
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Authors Affiliations
  1. 1. Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, AL, United States
  2. 2. Department of Pediatrics, Faculty of Medicine, Kuwait University, Safat, Kuwait
  3. 3. Research Center for Immunodefi ciencies, Department of Allergy and Immunology, Children’s Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Tehran, 14194, Iran
  4. 4. Department of Oncology, St. Jude Children’s Research Hospital, Memphis, 38105, TN, United States
  5. 5. Research Center for Immunodeficiencies, Children’s Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Dr Qarib St, Keshavarz Blvd, Tehran, 14194, Iran
  6. 6. Department of Immunology, School of Medicine, and Molecular Immunology Research Center, Tehran University of Medical Sciences, Dr Qarib St, Keshavarz Blvd, Tehran, 14194, Iran

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.
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