GASTRIC CANCER IS THE MOST LETHAL COMPLICATION IN CVID: DATA FROM ITALIAN PID CENTRES AND PROPOSAL FOR A NEW SCREENING ALGORITHM
- Federica Pulvirenti, Italy
Abstract
Background and Aims
CVID is a heterogeneous disorder characterized by hypogammaglobulinemia, poor vaccine responses, and defects of memory B-cells. In addition to increased susceptibility to sinopulmonary infections, autoimmunity and granulomatous disease, an increased prevalence of lymphoma and of gastric cancer (GC) has been observed. Risk factors and surveillance strategies for GC in CVID are unclear.
Methods
Overall and GC incidence rates were evaluated in 455 CVID patients regularly followed up in three Italian Centres from 1993 to 2017 and compared with age-adjusted incidence rates in the Italian Cancer Registry database.
Results
CVID patients showed an increased cancer incidence for all sites combined (Obs = 133, SIR = 2.4; 95%CI = 1.7–3.5), due to an excess of non-Hodgkin lymphoma (Obs = 33, SIR = 14.3; 95%CI = 8.4–22.6) and of GC (Obs = 25; SIR = 6.4; 95%CI = 3.2–12.5) (Fig.1). H.pylori was significantly related to GC (OR=5.3, 95%CI=1.1–24.8, p=0.042). In contrast to other reports, GC was the leading cause of death in CVID. Standardized mortality ratio indicated a 10.1-fold excess mortality in CVID with GC. CVID developed GC 15 years earlier than the normative population, but they had a similar overall survival. Only CVID diagnosed at early GC stage survived >24months (Fig.2). Stomach histology performed before cancer onset showed atrophic gastritis, intestinal metaplasia or dysplasia. CVID patients might progress rapidly to advanced cancer stage as shown by patients developing III-IV stage within 1 year from an endoscopy negative for dysplasia.
Conclusions
Based on this large case series of GC in CVID we proposed a new screening program for CVID