TY - JOUR. T1 - Enteropathy-associated T-cell lymphoma of the jejunum complicated with intestinal perforation. AU - Chen, Li Mien. AU - Fan, Yang G.. AU - Yiang, Yia Tang. AU - Chang, Chen C.. AU - Lee, Wei H.. PY - 2003/4/1. Y1 - 2003/4/1. N2 - Enteropathy-associated T-cell lymphoma (EATL) is a rare, well-documented complication of celiac disease, accounting for less than 1% of the non-Hodgkins lymphomas. Perforation as the presentation of intestinal lymphoma is rare, and as the presentation of EATL is even rarer. Herein, we report a 56-year-old female with EATL of the jejunum complicated with intestinal perforation. She was admitted because of sudden onset of severe abdominal pain. Emergent exploratory laparatomy was done under the impression of perforative peptic ulcer, however, an ulcerative tumor with perforation was noted unexpectedly at the proximal jejunum. After tumor resection and end-to-end anastomosis of the jejunum, the patient received eight courses of CHOP (cyclophosphamide, ...
Enteropathy-associated T-cell lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell lymphoma that affects the small intestine. It is the most common primary gastrointestinal T-cell lymphoma, arising from the T cells that are found between the cells that line the small intestinal (brush border cells or small intestinal epithelial cells). These cancerous T-cells are a possible consequence of refractory cases of coeliac disease or in chronic, untreated cases in genetically susceptible individuals. EATL can be classified as an extranodal peripheral T cell lymphoma, a category it shares with hepatosplenic T cell lymphoma and panniculitic T cell lymphoma. It can be further classified in type I and II EATL. Enteropathy associated T-cell lymphoma (EATL) is environmentally induced as a result of the consumption of Triticeae glutens (e.g. wheat gluten). In gluten-sensitive individuals with EATL, 68% are homozygotes of the DQB1*02 subtype at the HLA-DQB1 locus. (See Coeliac ...
Enteropathy-associated T-cell lymphoma (EATL), a rare and aggressive intestinal malignancy of intraepithelial T lymphocytes, comprises two disease variants (EATL-I and EATL-II) differing in clinical characteristics and pathological features. Here we report findings derived from whole exome sequencing of 15 EATL-II tumor-normal tissue pairs. The tumor suppressor gene SETD2 encoding a non-redundant H3K36-specific trimethyltransferase is altered in 14/15 cases (93%), mainly by loss-of-function mutations and/or loss of the corresponding locus (3p21.31). These alterations consistently correlate with defective H3K36 trimethylation. The JAK/STAT pathway comprises recurrent STAT5B (60%), JAK3 (46%) and SH2B3 (20%) mutations, including a STAT5B V712E activating variant. Additionally, frequent mutations in TP53, BRAF and KRAS are observed. Conversely, in EATL-I, no SETD2, STAT5B or JAK3 mutations are found, and H3K36 trimethylation is preserved. This study describes SETD2 inactivation as EATL-II molecular ...
Enteropathy refers to any pathology of the intestine. Although enteritis specifically refers to an inflammation of the intestine, and is thus a more specific term than enteropathy, the two phrases are sometimes used interchangeably. Specific types of enteropathy include: Enteropathy-associated T-cell lymphoma Environmental enteropathy An incompletely defined syndrome of inflammation related to the quality of the environment. Signs and symptoms include reduced absorptive capacity and reduced intestinal barrier function of the small intestine. It is widespread among children and adults in low- and middle-income countries. Eosinophilic enteropathy A condition in which eosinophils (a type of white blood cell) accumulate in the gastrointestinal tract and in the blood. Eosinophil build up in the gastrointestinal tract can result in polyp formation, tissue break down, inflammation, and ulcers. Gluten-sensitive enteropathy (which can progress to coeliac disease) Coeliac disease A malabsorption ...
Enteropathy-associated T cell lymphoma (EATL) is a lethal, and the most common, neoplastic complication of celiac disease. Here, we defined the genetic landscape of EATL through whole-exome sequencing of 69 EATL tumors. SETD2 was the most frequently silenced gene in EATL (32% of cases). The JAK-STAT pathway was the most frequently mutated pathway, with frequent mutations in STAT5B as well as JAK1, JAK3, STAT3, and SOCS1 We also identified mutations in KRAS, TP53, and TERT Type I EATL and type II EATL (monomorphic epitheliotropic intestinal T cell lymphoma) had highly overlapping genetic alterations indicating shared mechanisms underlying their pathogenesis. We modeled the effects of SETD2 loss in vivo by developing a T cell-specific knockout mouse. These mice manifested an expansion of γδ T cells, indicating novel roles for SETD2 in T cell development and lymphomagenesis. Our data render the most comprehensive genetic portrait yet of this uncommon but lethal disease and may inform future ...
Peripheral T-cell lymphoma, NOS. Angioimmunoblastic T-cell lymphoma. Anaplastic large cell lymphoma, ALK-positive. Anaplastic large cell lymphoma, ALK-negative. Enteropathy-associated T-cell lymphoma. Adult T-cell leukemia/lymphoma. Hydroa vacciniforme-like lymphoma. T-cell prolymphocytic leukemia. T-cell large granular lymphocytic leukemia. Hepatosplenic T-cell lymphoma. Extranodal NK/T-cell lymphoma, nasal type. Aggressive NK cell leukemia. Systemic EBV+ T-cell lymphoproliferative disease of childhood (associated with chronic active EBV infection). Chronic lymphoproliferative disorder of NK cells* ...
CT scan is a useful tool in discriminating between CD and (Pre) EATL. RCD II and EATL showed more bowel wall thickening, lymphadenopathy and intussusception, less increase in number of small mesenteric vessels and a smaller splenic volume compared with CD and RCD I.
Refractory celiac disease (known as RCD) is quite related, but yet different from celiac disease on its own, an increasingly common digestive disorder.
Sigma-Aldrich offers abstracts and full-text articles by [R L J van Wanrooij, D M J Müller, E A Neefjes-Borst, J Meijer, L G Koudstaal, D A M Heideman, H J Bontkes, B M E von Blomberg, G Bouma, C J J Mulder].
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Thats interesting about the intestinal parasite I have sometimes wondered if had something like that , but I havent really looked into it much , I went to the Caribbean 5 years ago and some problems started after that , its probably not connected but I have lately thought about if I did pick something up . My doctor ruled out refractory celiac disease in 2011 , I last had a endoscopy and biopsy at the end of 2010 , my villi was okay all the test showed was mild gastritis . Also my serology levels or test have always been normally , my gastro doctor did check again recently and said the tests were fine as usual indicating good control of the disease Its odd why I dont absorb Vitamin D very well , I need to go to a sunny country for a few months ! Can you still get Vitamin D when the sun is not out on cloudy and rainy days ? Sorry to hear you have suffered from depression for a over a decade I agree its best to find the reason , I want the problems to improve but not just by themselves just ...
We read with interest the article of Liu et al in Gut, in which the authors emphasise the need for monitoring of clonality and intraepithelial lymphocyte (IEL) immunophenotype in the surveillance of refractory coeliac disease (RCD).1. The authors state there is no consensus on the cut-off of aberrant cells distinguishing between non-complicated coeliac disease (CD), RCD type I (RCDI) and RCD type II (RCDII). However, in 2000 it was shown that based on the number of aberrant T cells, CD can be distinguished from RCD by immunohistochemistry.2 Furthermore, using flow cytometry, Verbeek defined a clinically well-validated cut-off of 20% IELs as being diagnostic for RCDII.3. In their paper, the authors describe that a high percentage of patients (80%) progress from RCDI to RCDII. This is somewhat … ...
Free, official coding info for 2021 ICD-10-CM C84.99 - includes detailed rules, notes, synonyms, ICD-9-CM conversion, index and annotation crosswalks, DRG grouping and more.
The quality of the soil at the transmission station sites plays a key role in the WATL and EATL construction project. Important characteristics of the soil on-site must be identified in order to determine its ability to support the transmission station structures. SGS soil testing is enabling project leaders to assess the suitability of the soil, providing them vital data to foster informed decision making and planning. SGS soil inspectors are completing laboratory testing for particle size distribution and compressive strengths, soil index and moisture/density. They are conducting software analysis and providing project partners technical support and consultancy. Concrete and aggregates used in the Canadian WATL and EATL construction project must also meet strict regulations and quality standards. Many standards are compulsory requiring independent third-party concrete and aggregate testing to help in assuring compliance. SGS is implementing the full range of its concrete and aggregate testing ...
The latest issue of American Journal of Gastroenterology reports on the diagnostic yield of capsule endoscopy in refractory celiac disease. ...
Nasal T/NK-cell lymphomas are highly associated with Epstein-Barr virus (EBV). They are more frequent in Asia than in Western countries. In Central and South America there are few studies about nasal T/NK-cell lymphoma and they have shown a strong predominance of this phenotype in Native American descents, supporting the hypothesis of a racial predisposition for the disease. We studied the lymphomas involving midline facial region at a Brazilian institution. T/NK cell lymphomas (16/25) were more frequently found compared to B lymphomas (9 cases, all B large cell). T/NK cell lymphomas involved predominantly the nasal region. Histologically they showed angioinvasion and necrosis. All of them were positive for CD3 and CD56 and showed numerous tumor cells labeled by EBER-1. Although disease was localized in 61% at diagnosis, there was no tendency to cure. The racial distribution of patients with T/NK-cell phenotype was similar to that found in B-cell lymphomas. EBV was more frequently found in ...
Calypso Biotech is a biotech company developing antibody therapies up to clinical proof-of-concept by leveraging its expertise in immunology and drug development. Calypso Biotechs portfolio consists of two therapeutic antibody programs planned to reach the clinic by 2016-2017 against gastro-intestinal auto-immune syndromes such as Crohns and Refractory Celiac diseases.. ...
Stephen M. Ansell, MD, PhD, provides a general overview of peripheral T-cell lymphoma and a retrospective outlook on the advancement of treatment.
Patients with persistent symptoms and/or villous atrophy despite strict adherence to a gluten-free diet (GFD) have non-responsive celiac disease (NRCD). A subset of these patients has refractory celiac disease (RCD), yet some NRCD patients may simply be reacting to gluten cross-contamination. Here we describe the effects of a 3-6 month diet of whole, unprocessed foods, termed the Gluten Contamination Elimination Diet (GCED), on NRCD. We aim to demonstrate that this diet reclassifies the majority of patients thought to have RCD type 1 (RCD1). We reviewed the records of all GFD-adherent NRCD patients cared for in our celiac center from 2005-2011 who were documented to have started the GCED. Response to the GCED was defined as being asymptomatic after the diet, with normal villous architecture on repeat biopsy, if performed. Prior to the GCED, all patients were interviewed by an experienced dietitian and no sources of hidden gluten ingestion were identified. 17 patients completed the GCED; 15 were female
TY - JOUR. T1 - Targeted mutational profiling of peripheral T-cell lymphoma not otherwise specified highlights new mechanisms in a heterogeneous pathogenesis. AU - Schatz, J. H.. AU - Horwitz, S. M.. AU - Teruya-Feldstein, J.. AU - Lunning, M. A.. AU - Viale, A.. AU - Huberman, K.. AU - Socci, N. D.. AU - Lailler, N.. AU - Heguy, A.. AU - Dolgalev, I.. AU - Migliacci, J. C.. AU - Pirun, M.. AU - Palomba, M. L.. AU - Weinstock, D. M.. AU - Wendel, H. G.. PY - 2015/1/10. Y1 - 2015/1/10. UR - http://www.scopus.com/inward/record.url?scp=84920670383&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84920670383&partnerID=8YFLogxK. U2 - 10.1038/leu.2014.261. DO - 10.1038/leu.2014.261. M3 - Letter. C2 - 25257991. AN - SCOPUS:84920670383. VL - 29. SP - 237. EP - 241. JO - Leukemia. JF - Leukemia. SN - 0887-6924. IS - 1. ER - ...