CVIDs may also present with characteristic non-infective complica

CVIDs may also present with characteristic non-infective complications.

PLX4720 Based on the complications, five distinct clinical phenotypes have been proposed: no disease-related complications, autoimmunity, polyclonal lymphocytic infiltration, enteropathy and lymphoid malignancy [3]. Dyspepsia occurs in at least 50% of the patients with CVIDs [4] and gastric pathology is found in about half of such patients [4]. Such pathology includes chronic or atrophic gastritis [5], lymphocytic or granulomatous gastritis [6], dysplasia [4], adenocarcinoma [4,6–10], mucosa-associated lymphoid tissue (MALT)-type lymphoma [11] or diffuse B cell lymphoma [12]. Besides a higher risk of lymphoma, patients with CVIDs also have a

10-fold increased risk of gastric cancer [10]. Following the first case of gastric cancer in a local cohort of 116 patients with CVIDs in 25 years, we review the risk factors for gastric cancer and report a cohort study of gastric pathology in these patients under long-term follow-up. We propose a surveillance protocol to improve and standardize the management of those CVID patients who have an increased risk of gastric malignancy. The aetiology of sporadic gastric cancer is multi-factorial, with contributions from genetic, lifestyle and environmental factors [13,14]. Non-modifiable risk factors include male gender, advancing age, genetic predisposition in some families, lower socio-economic status, blood group A and a past history of Epstein–Barr virus infection, radiation or gastric surgery. Modifiable risk factors include Selleckchem Roscovitine Helicobacter pylori infection, pernicious anaemia, diet (consumption of salt-preserved foods and N-nitroso compounds), smoking and geography [14]. Prognosis is generally poor and 5-year survival lies between 10 and 20% [14,15]. A population-based screening programme for gastric cancer, introduced in Japan in 1960, where the standardized

incidence rates of 69·2 per 105 in males and 28·6 per 105 in females compared to < 15 per 105 in western Europe, resulted in a 5-year survival rate of 60% [16]. This programme invites all individuals over the age of 40 years for an annual risk assessment and double-contrast 3-mercaptopyruvate sulfurtransferase barium study, with endoscopy if an abnormality is found. The standardized mortality rates for gastric cancer decreased from 70·7 to 21·9 in males and 37·1 to 8·4 in females between 1960 and 2006 ( [17]. Two cohort studies have also demonstrated reduced mortality from gastric cancer screening programmes, even when adjusted for confounding lifestyle measures. In 42 150 people followed for 13 years, deaths from gastric cancer halved with screening [relative risk (RR) 0·52; 95% confidence interval (CI) 0·36–0·74], due to a decreased incidence of advanced gastric cancer in the screened group (RR 0·75, 95% CI 0·58–0·96) [18].

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