Researchers find increased cancer incidence among patients with primary immunodeficiency disorders

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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 United States 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 will be presented at the American Society of Clinical Oncology (ASCO) 52nd Annual Meeting in Chicago.

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 through the United States, with data from the USIDNET registry. Cancer incidence was compared based on age and gender.

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 eight 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," says 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," adds first author Paul Mayor, MD, Fellow in the Department of Gynecologic Oncology at RPCI.

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