Abstract
An increased prevalence of malignant lymphoma and of gastric cancer has been observed in large cohorts of patients with common variable immunodeficiency (CVID), the most frequently symptomatic primary immunodeficiency. Surveillance strategies for cancers in CVID should be defined based on epidemiological data. Risks and mortality for cancers among 455 Italian patients with CVID were compared to cancer incidence data from the Italian Cancer Registry database. 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 gastric cancer (Obs = 25; SIR = 6.4; 95%CI = 3.2–12.5). CVID patients with gastric cancer and lymphoma had a worse survival in comparison to cancer-free CVID (HR: 4.8, 95%CI: 4.2–44.4 and HR: 4.2, 95%CI: 2.8–44.4). Similar to what observed in other series, CVID-associated lymphomas were more likely to be of B cell origin and often occurred at extra-nodal sites. We collected the largest case-series of gastric cancers in CVID subjects. In contrast to other reports, gastric cancer was the leading cause of death in CVID. Standardized mortality ratio indicated a 10.1-fold excess mortality among CVID patients with gastric cancer. CVID developed gastric cancer 15 years earlier than the normative population, but they had a similar overall survival. Only CVID diagnosed at early stage gastric cancer survived >24 months. Stomach histology from upper endoscopy performed before cancer onset showed areas of atrophic gastritis, intestinal metaplasia or dysplasia. CVID patients might progress rapidly to an advanced cancer stage as shown by patients developing a III-IV stage gastric cancer within 1 year from an endoscopy without signs of dysplasia. Based on high rate of mortality due to gastric cancer in Italian CVID patients, we hereby suggest a strategy aimed at early diagnosis, based on regular upper endoscopy and on Helicobacter pylori infection treatment, recommending an implementation of national guidelines.
Highlights
Inherited conditions affecting immune system function are classified as primary immune deficiencies (PID) [1]
Based on the high rate of mortality for gastric cancer in Italian common variable immunodeficiency (CVID) patients, we highlight the need of a strategy for an earlier diagnosis and we suggest a new schedule for gastric endoscopy in CVID patients
H. pylori status and histology of gastric endoscopic biopsies collected before the diagnosis of cancer was available for 7 patients (Table 7): areas of dysplasia were identified in two subjects whereas areas of atrophic gastritis and/or intestinal metaplasia were found in all patients
Summary
Inherited conditions affecting immune system function are classified as primary immune deficiencies (PID) [1]. Among PID, an increased prevalence of cancer is recognized in patients affected by common variable immunodeficiency (CVID), the most common symptomatic primary antibody defect. Our group described a higher prevalence of lymphoma and gastric carcinomas in the Italian cohort of CVID in comparison to the normative population [8]. Of lymphoma and gastric carcinomas in a four decades study showing that 21% of adult CVID patients developed cancers. We suggested that the discrepancy in cancer survival, between the two cohorts, might have been due to the high prevalence of deaths for malignancies other than lymphoma in the Italian CVID cohort, and to deaths for gastric cancer. We analyzed data on the prevalence of hematological and non-hematological malignancies, on cancer risk, on mortality and on survival rate in a cohort of 455 Italian adult CVID patients compared to normative population. Based on the high rate of mortality for gastric cancer in Italian CVID patients, we highlight the need of a strategy for an earlier diagnosis and we suggest a new schedule for gastric endoscopy in CVID patients
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