© ASCO/Zach Boyden-Holmes
CHICAGO—Investigators have found an increased risk in cancer incidence for patients with primary immunodeficiency diseases (PIDD), and in particular, a significant increase in lymphoma cases.
Investigators reviewed records of patients registered in the United States Immune Deficiency Network (USIDNET) and found they had a 42% increase in cancer incidence overall compared to the general population in the Surveillance, Epidemiology and End Results (SEER) database.
And lymphoma incidence was 10 times higher among men and 8 times higher among women in the USIDNET registry.
The USIDNET registry collects information, including clinical, laboratory, and outcome data, on patients affected by PIDD. Site-specific cancer incidence rates are included in the registry.
Investigators compared data from the 2 registries based on age and gender. They abstracted data on 3665 patients from the USIDNET Registry from 2003 to 2015 and generated site-specific incidence rates for them. They also generated age adjusted incidence rates using the SEER database for comparison.
The investigators observed a 1.34-fold excess relative risk of cancer (P<0.001) in patients with PIDD compared to the age-adjusted SEER population.
They also discovered that in men, the relative risk increased to 1.8-fold (P<0.001), while in women, the excess relative risk of cancer was not significant.
Men also had a statistically significant increase in skin cancer (4.45-fold excess relative risk, P<0.001) and thyroid cancer (4-fold excess relative risk, P=0.002).
Women had a statistically significant increase in skin (3.19-fold excess relative risk, P<0.001) and stomach cancer (3-fold excess relative risk, P=0.015).
And both men and women had a statistically significant increase in lymphoma, at a significance of P<0.001 for each gender.
“This study found that patients with primary immunodeficiency disorders have a modest increase in overall cancer incidence,” senior author, Brahm Segal, MD, of Roswell Park Cancer Institute in Buffalo, New York, said, “driven by specific primary immunodeficiency disorders predisposing to specific cancers, particularly lymphoma.”
The investigators did not observe an increased risk for the most common solid tumor cancers, including breast, lung, prostate, and colon.
The investigators believe the findings point to a “restricted role of the immune system in protecting from specific cancers and question the role of immunosurveillance in incident risk of common solid tumor cancers.”
They reported their findings at the 2016 ASCO Annual Meeting as abstract 1520.