Tailored Cancer Surveillance May be Warranted for Patients with Primary Immunodeficiency Diseases

A new study indicates that patients with primary immunodeficiency diseases may be at a much higher risk for certain cancers, especially lymphoma.

A new study is suggesting a restricted but important role for the immune system in protecting against certain types of cancer. In the largest study of its kind, researchers found a higher risk of some cancers in patients with immune deficiency; the strongest association was with lymphoma. The researchers found no significant increase in the four most common malignancies in men and women (lung, colon, breast, and prostate cancers). However, there were significant increases in lymphoma in both men (10-fold increase; P < .001) and women (8.34-fold increase, P < .001) with primary immunodeficiency diseases (PIDDs).

The findings, which were published online ahead of print in the Journal of Allergy and Clinical Immunology, suggest cancer prevention efforts may need to be much more tailored in this patient population. PIDD are a group of more than 300 disorders caused by single-gene defects that prevent the immune system from functioning properly. These immune disorders affect 1 in 1,200 people.

For this current investigation, researchers evaluated the incidence of cancer among patients with PIDD registered in the United States Immune Deficiency Network (USIDNET). This registry gathers information regarding the history and outcomes of patients with PIDD. The researchers compared rates of cancer in USIDNET patients with those of the general population using data from the Surveillance, Epidemiology and End Results Program (SEER). The investigation compared overall and site-specific rates of cancer in 3,658 PIDD patients and an age-adjusted SEER population.

Senior author Brahm Segal, MD, Chief of Infectious Diseases and Member of the Department of Immunology at Roswell Park Cancer Institute in Buffalo, New York, said the cancers observed with higher frequency in PIDD patients were generally associated with an infectious cause. He said these findings underscore the need for evidence-based approaches for early detection in high-risk PIDD patients.

“Our analysis points to the immune system having a restricted role in protecting us from cancer. Specific primary immunodeficiencies increase the risk of specific cancer, and usually these cancers are linked to viral infections, such as EBV [Epstein-Barr virus]-associated lymphomas. We're discussing follow-up studies with our colleagues,” Dr Segal told OncoTherapy Network.

The team found a 42% increase in overall cancer incidence among patients with PIDD. The majority of cancers (70%) were noted in patients with common variable immunodeficiency, followed by hypogammaglobulinemia and agammaglobulinemia (9%), and Wiskott-Aldrich syndrome (4.6%). The cancer types with the highest incidence rates among PIDD patients in the USIDNET registry were lymphoma, skin cancer, and thyroid cancer.