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.

Gastric cancer is the leading cause of death in Italian adult patients with common variable immunodeficiency / Pulvirenti, Federica; Pecoraro, Antonio; Cinetto, Francesco; Milito, Cinzia; Valente, Michele; Santangeli, Enrico; Crescenzi, Ludovica; Rizzo, Francesca; Tabolli, Stefano; Spadaro, Giuseppe; Agostini, Carlo; Quinti, Isabella. - In: FRONTIERS IN IMMUNOLOGY. - ISSN 1664-3224. - 9:(2018), pp. 1-12. [10.3389/fimmu.2018.02546]

Gastric cancer is the leading cause of death in Italian adult patients with common variable immunodeficiency

Pulvirenti, Federica;Milito, Cinzia
Investigation
;
Santangeli, Enrico;Spadaro, Giuseppe;Quinti, Isabella
Ultimo
Conceptualization
2018

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.
2018
Cancer; Common variable immunodeficiency; Gastric cancer; Guidelines; IgA; Lymphoma; Risk; Upper endoscopy; Immunology and Allergy; Immunology
01 Pubblicazione su rivista::01a Articolo in rivista
Gastric cancer is the leading cause of death in Italian adult patients with common variable immunodeficiency / Pulvirenti, Federica; Pecoraro, Antonio; Cinetto, Francesco; Milito, Cinzia; Valente, Michele; Santangeli, Enrico; Crescenzi, Ludovica; Rizzo, Francesca; Tabolli, Stefano; Spadaro, Giuseppe; Agostini, Carlo; Quinti, Isabella. - In: FRONTIERS IN IMMUNOLOGY. - ISSN 1664-3224. - 9:(2018), pp. 1-12. [10.3389/fimmu.2018.02546]
File allegati a questo prodotto
File Dimensione Formato  
Pulvirenti_gastric-cancer_2018.pdf

accesso aperto

Tipologia: Versione editoriale (versione pubblicata con il layout dell'editore)
Licenza: Tutti i diritti riservati (All rights reserved)
Dimensione 902.45 kB
Formato Adobe PDF
902.45 kB Adobe PDF

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1256377
Citazioni
  • ???jsp.display-item.citation.pmc??? 25
  • Scopus 53
  • ???jsp.display-item.citation.isi??? 49
social impact