Mesenteric lymph node cavitation in celiac disease: Report of four cases and literature review.

CONCLUSION: The severity of this complication deserves to be known and should lead to its research in celiac patients, especially in cases diagnosed in adulthood or in case of refractory disease. PMID: 31155369 [PubMed - as supplied by publisher]
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research

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Authors: Lenti MV, Biagi F, Lucioni M, Di Sabatino A, Paulli M, Corazza GR Abstract Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an aggressive type of intestinal lymphoma which affects individuals without evidence of enteropathy. In this single-centre case series, we describe the first two cases of MEITL in Caucasian patients suffering from histologically-proven coeliac disease (CD). Original medical records were retrieved and anonymised. All biopsy and surgical MEITL specimens were reviewed by three haematopathologists. Two patients aged 63- and 55-year old at CD diagnosis, subsequently develo...
Source: Scandinavian Journal of Gastroenterology - Category: Gastroenterology Tags: Scand J Gastroenterol Source Type: research
We report a 54-year-old Caucasian man who presented with chronic diarrhoea and weight loss. He was diagnosed with coeliac disease based on positive serology results and duodenal, jejunal, and ileal biopsies that showed villous atrophy. Despite adherence to a gluten-free diet, there was no clinical remission and enteropathy-associated T cell lymphoma was suspected. Repeated endoscopic biopsy showed persistent mucosal disease but no evidence of lymphoma. Several weeks later he presented with a perforated jejunum. Histology of the resected jejunum showed diffuse infiltration of submucosa and muscularis propria by malignant ly...
Source: Malaysian Journal of Pathology - Category: Pathology Tags: Malays J Pathol Source Type: research
Abstract Celiac disease (CD) is the most common autoimmune enteropathy worldwide. In CD, dietary gluten triggers a T cell driven small intestinal inflammation in a subset of genetically predisposed subjects, expressing the HLA DQ2 and/or DQ8 genes on their antigen presenting cells. HLA DQ2/DQ8 can bind gluten peptides after their prior modification by the CD autoantigen, tissue transglutaminase (TG2). This process leads to the activation of gluten reactive T cells, small bowel villous atrophy, crypt hyperplasia and intraepithelial lymphocytosis, the histological hallmarks of CD. The clinical picture of CD is extre...
Source: European Journal of Internal Medicine - Category: Internal Medicine Authors: Tags: Eur J Intern Med Source Type: research
Conclusion: Although most patients had limited stage, the clinical outcomes of MEITL patients were dismal. While the optimal management of MEITL remains undetermined, achieving CR and consolidative ASCT seem to be essential. In addition, considering the frequent local failure, as well as the CNS relapse, novel therapeutic approaches are required to improve survival.Figure.DisclosuresKim: Mundipharma: Research Funding; Merck: Research Funding; Celgene: Research Funding; Takeda: Research Funding; Roche: Honoraria, Research Funding; Kyowa-Kirin: Research Funding; Eisai: Honoraria, Research Funding; J&J: Research Funding; Cell...
Source: Blood - Category: Hematology Authors: Tags: 624. Hodgkin Lymphoma and T/NK Cell Lymphoma-Clinical Studies: Poster II Source Type: research
Publication date: Available online 28 September 2018Source: The Journal of Molecular DiagnosticsAuthor(s): Coralie Derrieux, Amélie Trinquand, Julie Bruneau, Virginie Verkarre, Ludovic Lhermitte, Marion Alcantara, Patrick Villarese, Bertrand Meresse, David Sibon, Olivier Hermine, Nicole Brousse, Thierry Molina, Christophe Cellier, Nadine Cerf-Bensussan, Georgia Malamut, Elizabeth MacintyreAbstractCeliac disease is a chronic inflammation of the small intestine with villous atrophy that can become refractory to a gluten-free diet. Two categories of refractory celiac disease (I and II) can be distinguished by the pheno...
Source: The Journal of Molecular Diagnostics - Category: Pathology Source Type: research
AbstractPurpose of ReviewTo provide an update on the pathogenesis of enteropathy-associated T cell lymphoma (EATL) and its relationship with refractory celiac disease (RCD), in light of current knowledge of immune, genetic, and environmental factors that promote neoplastic transformation of intraepithelial lymphocytes (IELs).Recent FindingsEATL frequently evolves from RCD type II (RCD II) but can occur “de novo” in individuals with celiac disease. Recurrent activating mutations in members of the JAK/STAT pathway have been recently described in EATL and RCD II, which suggests deregulation of cytokine signaling t...
Source: Current Hematologic Malignancy Reports - Category: Hematology Source Type: research
Background Approximately 5% of patients with celiac disease (CeD) do not respond to a gluten-free diet and progress to refractory celiac disease (RCD), a severe progression that is characterized by infiltration of intraepithelial T lymphocytes. Patients with RCD type II (RCDII) show clonal expansions of intraepithelial T lymphocytes that result in a poor prognosis and a high mortality rate through development of aggressive enteropathy-associated T-cell lymphoma. It is not known whether genetic variations play a role in severe progression of CeD to RCDII. Patients and methods We performed the first genome-wide associat...
Source: European Journal of Gastroenterology and Hepatology - Category: Gastroenterology Tags: Original Articles: Gastroenterology Source Type: research
AbstractPurpose of ReviewTo provide an update on the pathogenesis of enteropathy-associated T cell lymphoma (EATL) and its relationship with refractory celiac disease (RCD), in light of current knowledge of immune, genetic, and environmental factors that promote neoplastic transformation of intraepithelial lymphocytes (IELs).Recent FindingsEATL frequently evolves from RCD type II (RCD II) but can occur “de novo” in individuals with celiac disease. Recurrent activating mutations in members of the JAK/STAT pathway have been recently described in EATL and RCD II, which suggests deregulation of cytokine signaling t...
Source: Current Hematologic Malignancy Reports - Category: Hematology Source Type: research
Authors: Collin P, Salmi TT, Hervonen K, Kaukinen K, Reunala T Abstract Dermatitis herpetiformis (DH) is an itchy blistering skin disease with predilection sites on elbows, knees, and buttocks. Diagnosis is confirmed by showing granular immunoglobulin A deposits in perilesional skin. DH is one manifestation of coeliac disease; the skin symptoms heal with gluten free diet (GFD) and relapse on gluten challenge. Of the first-degree relatives, 5% may be affected by either condition. Tissue transglutaminase (TG2) is the autoantigen in coeliac disease and epidermal transglutaminase (TG3) in DH. Both diseases conditions e...
Source: Annals of Medicine - Category: Internal Medicine Tags: Ann Med Source Type: research
CONCLUSION: These findings showed that in contrast to classical enteropathy-associated T-cell lymphoma, where the small bowel is the exclusive primary site (owing to its origin from coeliac disease) and distant metastases even during relapse are exceptional, MEITL might on presentation and during relapse involve any part of the gut, and metastasize to multiple extra-intestinal sites. PMID: 28581364 [PubMed - as supplied by publisher]
Source: Hematology - Category: Hematology Tags: Hematology Source Type: research
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