Effect of a Gluten-free Diet on the Risk of Enteropathy-associated T-cell Lymphoma in Celiac Disease

Silano, Marco; Volta, Umberto; Vincenzi, A. De; Dessı̀, Mariarita; Vincenzi, Massimo De

doi:10.1007/s10620-007-9952-8

Cited by 79 publications

(41 citation statements)

References 20 publications

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“…3 This association is particularly clear in the case of a specific nonHodgkin lymphoma (NHL) subtype, enteropathy-associated T cell lymphoma (EATL), which has been long established as a complication of coeliac disease. 4 The magnitude of the risk of other forms of lymphoid malignancies is much less clear. Data on lymphoid malignancy risk other than NHL are scant, and the risk estimates for the more commonly reported NHL vary significantly.…”

Section: Introductionmentioning

confidence: 99%

Meta‐analysis: coeliac disease and the risk of all‐cause mortality, any malignancy and lymphoid malignancy

Tio

Cox

Eslick

2012

Aliment Pharmacol Ther

124

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Section: Introductionmentioning

confidence: 99%

Meta‐analysis: coeliac disease and the risk of all‐cause mortality, any malignancy and lymphoid malignancy

Tio

Cox

Eslick

2012

Aliment Pharmacol Ther

124

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“…The small bowel lymphoma risk in CD significantly decreases with successful treatment (26). This may not be the case for the risk of SBA in CD.…”

Section: ------------------------------------------------------------mentioning

confidence: 95%

Small bowel adenocarcinomas in celiac disease follow the CIM-MSI pathway

et al. 2010

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Abstract. Celiac disease (CD) is an inflammatory disorder associated with an increased risk of small bowel adenocarcinoma. Recent studies have demonstrated aberrant CpG island methylation (CIM) in chronic inflammation, aging and cancer. We hypothesized that CIM may link CD to small bowel carcinogenesis. We determined microsatellite instability (MSI), CIM, and expression of MLH1 and MGMT in 3 CDassociated small bowel carcinomas and corresponding nonneoplastic mucosa. The results were compared to those of small bowel mucosa from CD patients without carcinoma and 20 small bowel carcinomas from a non-CD origin. A high level CIM/MSI phenotype was found in all of the 3 CDassociated carcinomas and was associated with loss of MLH1 expression due to hypermethylation of the MLH1 promoter. This phenotype was noted in only 2 of the 20 investigated non-CD-associated carcinomas. Low-level CIM was already detectable in 9 of the 12 non-neoplastic mucosa samples of CD patients and in non-CD-associated carcinomas of elderly patients. In conclusion, our data reveal that the high-level CIM/MSI pathway is typical of CD-associated small bowel carcinomas and indicate that aberrant CpG island methylation links CD and carcinogenesis. The data further suggest that CD should be considered in patients with small bowel adenocarcinoma, particularly when the tumors display MSI.

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“…[84][85][86][87][88][89][90][91] However, on the basis of a reduced malignancy risk in asymptomatic celiac patients diagnosed because of familiarity compared with celiac patients diagnosed because of symptoms, it has recently been suggested that there is no evidence that a GFD further reduces the risk of malignant complications in asymptomatic celiac patients. 92 Until these results are confirmed, we strongly recommend that all celiac patients must maintain a strict life-long GFD.…”

Section: Prophylaxis: the Role Of A Gluten-free Dietmentioning

confidence: 99%

How I treat enteropathy-associated T-cell lymphoma

Sabatino

Biagi

Gobbi

et al. 2012

Blood

100

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Enteropathy-associated T-cell lymphoma (EATL) is a complication of celiac disease (CD). This tumor derives from the neoplastic transformation of aberrant intraepithelial T lymphocytes emerging in celiac patients unresponsive to a gluten-free diet. Poor adherence to a gluten-free diet, HLA-DQ2 homozygosity, and late diagnosis of CD are recognized as risk factors for malignant evolution of CD. Recurrence of diarrhea, unexplained weight loss, abdominal pain, fever, and night sweating should alert physicians to this complication. The suspicion of EATL should lead to an extensive diagnostic workup in which magnetic resonance enteroclysis, positron emission tomography scan, and histologic identification of lesions represent the best options. Treatment includes high-dose chemotherapy preceded by surgical resection and followed by autologous stem cell transplantation, although biologic therapies seem to be promising. Strict adherence to a gluten-free diet remains the only way to prevent EATL. (Blood. 2012;119(11): 2458-2468) IntroductionCeliac disease (CD) is a chronic gluten-sensitive enteropathy characterized by a high prevalence in the general population and an increased mortality. 1,2 It is well known that the increased mortality is mainly the result of the complications of CD itself, represented by refractory CD (RCD) and enteropathy-associated T-cell lymphoma (EATL). 2 RCD is a form of CD that does not respond histologically to at least 12 months of a strict gluten-free diet (GFD). 3 RCD can also evolve in patients who initially responded normally to a GFD and who are still maintaining a strict GFD. On the basis of the intraepithelial lymphocyte (IEL) population, RCD is further classified into type 1 and type 2. RCD type 1 (RCD1) is characterized by persisting villous atrophy despite a strict GFD associated with increased but still phenotypically normal IELs. Conversely, a clonal expansion of abnormal IELs lacking surface CD3, CD8, and T-cell receptor (TCR) markers but expressing intracellular CD3 indicates RCD type 2 (RCD2), a condition that frequently evolves into EATL, the most serious complication of CD. 4 In the past, up to 20% of celiac patients were considered to be affected by a nonresponsive form of CD. This was obviously because of the lack of consensus on the definition of nonresponsiveness. 4 In the last few years, some evidence has suggested that the prevalence of RCD is low, ranging from 0.6% to 1.5%. 3,5,6 Although the presence of RCD in CD is probably to be approximately 1%, the prevalence of RCD2 among patients with RCD is far from being ascertained. Probably because of not only the great differences in the diagnostic criteria, 4 but also referral bias, different papers report very different results, which vary from 15% to 75%. 7-9 Finally, although the incidence of EATL was reported to be rare in the general population (1 per million person-years), 10,11 it was shown that it occurs in 60% to 80% of patients with RCD2 within 5 years. [7][8][9]12 The description of EATL arising in patients with RCD...

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Effect of a Gluten-free Diet on the Risk of Enteropathy-associated T-cell Lymphoma in Celiac Disease

Cited by 79 publications

References 20 publications

Meta‐analysis: coeliac disease and the risk of all‐cause mortality, any malignancy and lymphoid malignancy

Meta‐analysis: coeliac disease and the risk of all‐cause mortality, any malignancy and lymphoid malignancy

Small bowel adenocarcinomas in celiac disease follow the CIM-MSI pathway

How I treat enteropathy-associated T-cell lymphoma

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