Cancer in primary immunodeficiency diseases: Cancer incidence in the United States Immune Deficiency Network Registry

Paul C. Mayor, Kevin H. Eng, Kelly L. Singel, Scott I. Abrams, Kunle Odunsi, Kirsten B. Moysich, Ramsay Fuleihan, Elizabeth Garabedian, Patricia Lugar, Hans D. Ochs, Francisco A. Bonilla, Rebecca H. Buckley, Kathleen E. Sullivan, Zuhair K. Ballas, Charlotte Cunningham-Rundles, Brahm H. Segal*

*Corresponding author for this work

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

Background: We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared with age-adjusted cancer incidence in the Surveillance, Epidemiology and End Results Program (SEER) database. Objective: We hypothesized that subjects with PIDD would have an increased incidence of cancer due to impaired immune function. Methods: Overall and site-specific cancer incidence rates were evaluated in subjects with PIDD (n = 3658) enrolled in the USIDNET registry from 2003 to 2015 and compared with age-adjusted incidence rates in the SEER database. Results: We observed a 1.42-fold excess relative risk of cancer in subjects with PIDD compared with the age-adjusted SEER population (P <.001). Men with PIDD had a 1.91-fold excess relative risk of cancer compared with the age-adjusted male population (P <.001), while women with PIDD had similar overall cancer rates compared with the age-adjusted female population. Of the 4 most common malignancies in men and women in SEER (lung, colon, breast, and prostate cancers), we found no significant increase in these diagnoses in subjects with PIDD. Significant increases in lymphoma in both men (10-fold increase, P <.001) and women (8.34-fold increase, P <.001) with PIDD were observed. Conclusions: Excess incidence of cancer occurred in subjects with PIDD. An excess of lymphoma in specific PIDD populations principally drove this increased incidence, while no increased risk of the most common solid tumor malignancies was observed. These data point to a restricted role of the immune system in protecting from specific cancers.

Original languageEnglish (US)
Pages (from-to)1028-1035
Number of pages8
JournalJournal of Allergy and Clinical Immunology
Volume141
Issue number3
DOIs
StatePublished - Mar 1 2018

Fingerprint

Registries
Incidence
SEER Program
Neoplasms
Population
Lymphoma
Databases
Colonic Neoplasms
Immune System
Lung Neoplasms
Prostatic Neoplasms
Breast Neoplasms

Keywords

  • Cancer
  • USIDNET
  • common variable immunodeficiency
  • leukemia
  • lymphoma
  • primary immunodeficiency disease

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Mayor, Paul C. ; Eng, Kevin H. ; Singel, Kelly L. ; Abrams, Scott I. ; Odunsi, Kunle ; Moysich, Kirsten B. ; Fuleihan, Ramsay ; Garabedian, Elizabeth ; Lugar, Patricia ; Ochs, Hans D. ; Bonilla, Francisco A. ; Buckley, Rebecca H. ; Sullivan, Kathleen E. ; Ballas, Zuhair K. ; Cunningham-Rundles, Charlotte ; Segal, Brahm H. / Cancer in primary immunodeficiency diseases : Cancer incidence in the United States Immune Deficiency Network Registry. In: Journal of Allergy and Clinical Immunology. 2018 ; Vol. 141, No. 3. pp. 1028-1035.
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title = "Cancer in primary immunodeficiency diseases: Cancer incidence in the United States Immune Deficiency Network Registry",
abstract = "Background: We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared with age-adjusted cancer incidence in the Surveillance, Epidemiology and End Results Program (SEER) database. Objective: We hypothesized that subjects with PIDD would have an increased incidence of cancer due to impaired immune function. Methods: Overall and site-specific cancer incidence rates were evaluated in subjects with PIDD (n = 3658) enrolled in the USIDNET registry from 2003 to 2015 and compared with age-adjusted incidence rates in the SEER database. Results: We observed a 1.42-fold excess relative risk of cancer in subjects with PIDD compared with the age-adjusted SEER population (P <.001). Men with PIDD had a 1.91-fold excess relative risk of cancer compared with the age-adjusted male population (P <.001), while women with PIDD had similar overall cancer rates compared with the age-adjusted female population. Of the 4 most common malignancies in men and women in SEER (lung, colon, breast, and prostate cancers), we found no significant increase in these diagnoses in subjects with PIDD. Significant increases in lymphoma in both men (10-fold increase, P <.001) and women (8.34-fold increase, P <.001) with PIDD were observed. Conclusions: Excess incidence of cancer occurred in subjects with PIDD. An excess of lymphoma in specific PIDD populations principally drove this increased incidence, while no increased risk of the most common solid tumor malignancies was observed. These data point to a restricted role of the immune system in protecting from specific cancers.",
keywords = "Cancer, USIDNET, common variable immunodeficiency, leukemia, lymphoma, primary immunodeficiency disease",
author = "Mayor, {Paul C.} and Eng, {Kevin H.} and Singel, {Kelly L.} and Abrams, {Scott I.} and Kunle Odunsi and Moysich, {Kirsten B.} and Ramsay Fuleihan and Elizabeth Garabedian and Patricia Lugar and Ochs, {Hans D.} and Bonilla, {Francisco A.} and Buckley, {Rebecca H.} and Sullivan, {Kathleen E.} and Ballas, {Zuhair K.} and Charlotte Cunningham-Rundles and Segal, {Brahm H.}",
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Mayor, PC, Eng, KH, Singel, KL, Abrams, SI, Odunsi, K, Moysich, KB, Fuleihan, R, Garabedian, E, Lugar, P, Ochs, HD, Bonilla, FA, Buckley, RH, Sullivan, KE, Ballas, ZK, Cunningham-Rundles, C & Segal, BH 2018, 'Cancer in primary immunodeficiency diseases: Cancer incidence in the United States Immune Deficiency Network Registry', Journal of Allergy and Clinical Immunology, vol. 141, no. 3, pp. 1028-1035. https://doi.org/10.1016/j.jaci.2017.05.024

Cancer in primary immunodeficiency diseases : Cancer incidence in the United States Immune Deficiency Network Registry. / Mayor, Paul C.; Eng, Kevin H.; Singel, Kelly L.; Abrams, Scott I.; Odunsi, Kunle; Moysich, Kirsten B.; Fuleihan, Ramsay; Garabedian, Elizabeth; Lugar, Patricia; Ochs, Hans D.; Bonilla, Francisco A.; Buckley, Rebecca H.; Sullivan, Kathleen E.; Ballas, Zuhair K.; Cunningham-Rundles, Charlotte; Segal, Brahm H.

In: Journal of Allergy and Clinical Immunology, Vol. 141, No. 3, 01.03.2018, p. 1028-1035.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Cancer in primary immunodeficiency diseases

T2 - Cancer incidence in the United States Immune Deficiency Network Registry

AU - Mayor, Paul C.

AU - Eng, Kevin H.

AU - Singel, Kelly L.

AU - Abrams, Scott I.

AU - Odunsi, Kunle

AU - Moysich, Kirsten B.

AU - Fuleihan, Ramsay

AU - Garabedian, Elizabeth

AU - Lugar, Patricia

AU - Ochs, Hans D.

AU - Bonilla, Francisco A.

AU - Buckley, Rebecca H.

AU - Sullivan, Kathleen E.

AU - Ballas, Zuhair K.

AU - Cunningham-Rundles, Charlotte

AU - Segal, Brahm H.

PY - 2018/3/1

Y1 - 2018/3/1

N2 - Background: We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared with age-adjusted cancer incidence in the Surveillance, Epidemiology and End Results Program (SEER) database. Objective: We hypothesized that subjects with PIDD would have an increased incidence of cancer due to impaired immune function. Methods: Overall and site-specific cancer incidence rates were evaluated in subjects with PIDD (n = 3658) enrolled in the USIDNET registry from 2003 to 2015 and compared with age-adjusted incidence rates in the SEER database. Results: We observed a 1.42-fold excess relative risk of cancer in subjects with PIDD compared with the age-adjusted SEER population (P <.001). Men with PIDD had a 1.91-fold excess relative risk of cancer compared with the age-adjusted male population (P <.001), while women with PIDD had similar overall cancer rates compared with the age-adjusted female population. Of the 4 most common malignancies in men and women in SEER (lung, colon, breast, and prostate cancers), we found no significant increase in these diagnoses in subjects with PIDD. Significant increases in lymphoma in both men (10-fold increase, P <.001) and women (8.34-fold increase, P <.001) with PIDD were observed. Conclusions: Excess incidence of cancer occurred in subjects with PIDD. An excess of lymphoma in specific PIDD populations principally drove this increased incidence, while no increased risk of the most common solid tumor malignancies was observed. These data point to a restricted role of the immune system in protecting from specific cancers.

AB - Background: We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared with age-adjusted cancer incidence in the Surveillance, Epidemiology and End Results Program (SEER) database. Objective: We hypothesized that subjects with PIDD would have an increased incidence of cancer due to impaired immune function. Methods: Overall and site-specific cancer incidence rates were evaluated in subjects with PIDD (n = 3658) enrolled in the USIDNET registry from 2003 to 2015 and compared with age-adjusted incidence rates in the SEER database. Results: We observed a 1.42-fold excess relative risk of cancer in subjects with PIDD compared with the age-adjusted SEER population (P <.001). Men with PIDD had a 1.91-fold excess relative risk of cancer compared with the age-adjusted male population (P <.001), while women with PIDD had similar overall cancer rates compared with the age-adjusted female population. Of the 4 most common malignancies in men and women in SEER (lung, colon, breast, and prostate cancers), we found no significant increase in these diagnoses in subjects with PIDD. Significant increases in lymphoma in both men (10-fold increase, P <.001) and women (8.34-fold increase, P <.001) with PIDD were observed. Conclusions: Excess incidence of cancer occurred in subjects with PIDD. An excess of lymphoma in specific PIDD populations principally drove this increased incidence, while no increased risk of the most common solid tumor malignancies was observed. These data point to a restricted role of the immune system in protecting from specific cancers.

KW - Cancer

KW - USIDNET

KW - common variable immunodeficiency

KW - leukemia

KW - lymphoma

KW - primary immunodeficiency disease

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U2 - 10.1016/j.jaci.2017.05.024

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