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ASCO 2016: Cancer Diagnosed More Often in Patients With Primary Immunodeficiency Disease

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Key Points

  • Researchers found a 42% increase in cancer incidence overall among patients within the USIDNET registry compared to the general population in the SEER database.
  • Lymphoma incidence was 10 times higher among men and 8 times higher among women in the USIDNET registry.
  • Men with a primary immunodeficiency disorder were more likely to be diagnosed with skin and thyroid cancer; women with a primary immunodeficiency disorder were more likely to be diagnosed with skin and stomach cancer.

Primary immunodeficiency disorders are a group of more than 300 single gene defects that affect the role of the immune system and prevent it from functioning properly. When Roswell Park Cancer Institute (RPCI) researchers evaluated the overall and site-specific incidence of cancer among patients registered in the U.S. Immune Deficiency Network (USIDNET), they found increased cancer incidence rates among patients with primary immunodeficiency diseases—and, in particular, a significant increase in lymphoma cases. The team’s findings were presented by Mayor et al at the 2016 ASCO Annual Meeting (Abstract 1520).

The USIDNET registry gathers variables including clinical, laboratory, and outcome data, which together provide a health survey of the relatively small number of patients affected by primary immunodeficiency disorders. The registry, believed to be the largest of its kind, is a repository for 39 academic institutions that have Institutional Review Board–approved protocols. Site-specific cancer incidence rates are included in the registry. This study compared data from the Surveillance, Epidemiology and End Results Program (SEER) of the National Cancer Institute, a population-based registry collecting cancer incidence and survival data from 18 geographic areas in the United States, with data from the USIDNET registry. Cancer incidence was compared based on age and gender.

Study Findings

The Roswell Park team found a 42% increase in cancer incidence overall among patients within the USIDNET registry compared to the general population in the SEER database. Lymphoma incidence was 10 times higher among men and 8 times higher among women in the USIDNET registry. Additionally, men with a primary immunodeficiency disorder were more likely to be diagnosed with skin and thyroid cancer; women with a primary immunodeficiency disorder were more likely to be diagnosed with skin and stomach cancer.

“This study found that patients with primary immunodeficiency disorders have a modest increase in overall cancer incidence. This increased incidence was driven by specific primary immunodeficiency disorders predisposing to specific cancers, particularly lymphoma. There was no observed increase risk in the most common cancers—cancers of the breast, lung, prostate, and colon,” said senior author Brahm Segal, MD, Chief of Infectious Diseases at Roswell Park.

“These data point to a restricted role of the immune system in protecting against specific cancers. One of the many functions of the immune system is to recognize cancerous or precancerous cells in the body in order to prevent cancer from developing. This study adds to the discussion about the role of this immunosurveillance in the risk of developing the common cancers among those with compromised immune systems,” added first author Paul Mayor, MD, Fellow in the Department of Gynecologic Oncology at RPCI.

The content in this post has not been reviewed by the American Society of Clinical Oncology, Inc. (ASCO®) and does not necessarily reflect the ideas and opinions of ASCO®.


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