Incidence of enteropathy-associated T-cell lymphoma in celiac disease: implications for children and adolescents with type 1 diabetes

Abstract

The long-term consequences of screening for celiac disease in diabetic children are not known. Routine screening is not practiced in our pediatric diabetic population. This study of the incidence of the most severe and specific long-term complication of untreated celiac disease, i.e., enteropathy-associated T-cell lymphoma (EATCL) and its association with diabetes, is done in order to justify our strategy not to practice routine screening. In the first phase of this study, a questionnaire was sent to all Swiss pathologists. The second phase consisted of a search in the cancer registry of the canton of Zurich. The incidence of EATCL in the general population of a Swiss region and the theoretical risk for a diabetic patient to develop this type of lymphoma were calculated. Ten cases of EATCL were found. Five had a long history of malabsorption, three of them since childhood. The mean age of the patients was 61.9 yr. None suffered from diabetes mellitus. The incidence of EATCL was 0.07/100,000 inhabitants/year. The expected risk for EATCL in patients with type 1 diabetes is 12.4/100,000 diabetic patients over a period of 60 yr. The data suggest that the risk for EATCL is small in diabetic patients. Therefore, we restrict the investigation for celiac disease to patients with typical and atypical symptoms, but do not perform routine screening.

Coeliac disease in children with Type 1 diabetes mellitus: the effect of the gluten‐free diet

“…The risk of malignancy in silent or subclinical disease is currently unclear [24]. It seems to be very low in diabetic patients [25]. As long as these potential risks have not been excluded, a lifelong gluten-free diet should be recommended to diabetic children with coeliac disease.…”

Celiac Disease and Pediatric Type 1 Diabetes: Diagnostic and Treatment Dilemmas

“…It remains unclear whether asymptomatic patients experience long- and/or short-term health related benefits from following a GFD [38, 66]. To our knowledge, no clinical studies have followed the natural progression of CD in patients with asymptomatic CD, and any beneficial effects of strict compliance with a GFD in asymptomatic patients with CD and T1D remain unknown.…”

“…Investigators identified 10 cases of EATCL in the Swiss population. Five patients had a history of malabsorption; however, none had T1D [66]. Investigators reported that the expected risk for EATCL is 12.4/100 000 in patients with T1D over a 60-year period and suggested that the risk of developing EATCL alone is not high enough to warrant routine screening for CD in T1D [66].…”

Coexistance of celiac disease and type 1 diabetes

“…In many studies there were no changes found in the level of HbA 1c and the demand for insulin after introducing a gluten-free diet [11, 54–57]. The results of the studies are also discrepant as regards the positive effect of dietary treatment on physical development parameters (height, body mass, body mass index) [11, 54–59] (Table III). …”

“…The risk of this kind of tumour is 50 times higher in the population of CD patients than in the overall population. The only study published so far on this issue did not confirm any increased risk of this type of lymphoma in patients with T1 diabetes mellitus [59]. Also, there is no data in medical literature concerning the impact of T1 diabetes mellitus on well-documented complications of untreated CD, such as disturbances in reproduction and increased mortality risk [39].…”