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Primary Immunodeficiencies and Cancers

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Cancer Immunology

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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 4–25 %, 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.

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Authors and Affiliations

  1. Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA

    Mona Hedayat MD

  2. Department of Pediatrics, Faculty of Medicine, Kuwait University, Safat, Kuwait

    Waleed Al-Herz

  3. Research Center for Immunodeficiencies, Department of Allergy and Immunology, Children’s Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Tehran, 14194, Iran

    Asghar Aghamohammadi MD, PhD

  4. Department of Oncology, St. Jude Children’s Research Hospital, Memphis, TN, 38105, USA

    Kim E. Nichols MD

  5. Department of Immunology, Research Center for Immunodeficiencies, Children’s Medical Center, Pediatrics Center of Excellence and Molecular Immunology Research Center, Tehran University of Medical Sciences, Dr Qarib St, Keshavarz Blvd, Tehran, 14194, Iran

    Nima Rezaei MD, MSc, PhD

  6. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Dr Qarib St, Keshavarz Blvd, Tehran, 14194, Iran

    Nima Rezaei MD, MSc, PhD

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  1. Mona Hedayat MD
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    Nima Rezaei

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Hedayat, M., Al-Herz, W., Aghamohammadi, A., Nichols, K.E., Rezaei, N. (2015). Primary Immunodeficiencies and Cancers. In: Rezaei, N. (eds) Cancer Immunology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-44006-3_18

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