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Enteropathy Associated T Cell Lymphoma

Sakura Tomita, Yara Y Kikuti, Joaquim Carreras Esteban, Katsuyoshi Takata, Tadashi Yoshino, Sílvia Beà, Elias Campo, Naoya Nakamura
No abstract text is available yet for this article.
February 2016: Pathology
Amy Webster, Philip Crea, Mark W Bamford, Roger Hew, Yvette Griffin, Fiona Miall
No abstract text is available yet for this article.
October 21, 2016: British Journal of Haematology
Zachary T Rivers, Drew R Oostra, James S Westholder, Gregory M Vercellotti
BACKGROUND: Romidepsin is a novel histone deacetylase inhibitor that is approved for the treatment of cutaneous and peripheral T-cell lymphoma in patients who have had at least one prior therapy. Romidepsin is generally well tolerated, though it comes with a risk of cardiac toxicities. OBJECTIVE: We report a case of electrocardiogram changes in a 64-year-old male with enteropathy-associated T-cell lymphoma, type 2, treated with salvage romidepsin therapy who relapsed after non-myeloablative allogeneic sibling peripheral blood stem cell transplant...
October 5, 2016: Journal of Oncology Pharmacy Practice
Yvette H van Beurden, Tom van Gils, Nienke A van Gils, Zain Kassam, Chris J J Mulder, Nieves Aparicio-Pagés
Treatment of refractory celiac disease type II (RCD II) and preventing the development of an enteropathy associated T-cell lymphoma in these patients is still difficult. In this case report, we describe a patient with RCD II who received fecal microbiota transfer as treatment for a recurrent Clostridium difficile infection, and remarkably showed a full recovery of duodenal villi and disappearance of celiac symptoms. This case suggests that altering the gut microbiota may hold promise in improving the clinical and histological consequences of celiac disease and/or RCD II...
September 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
Wei Guan, Quan-Shi Wang, Hu-Bing Wu, Wen-Lan Zhou
OBJECTIVE: To investigate the characteristic (18)F-FDG PET/CT findings in patients with primary intestinal lymphoma (PIL). METHODS: We collected the clinical and (18)F-FDG PET/CT data of 23 patients with PIL who underwent (18)F-FDG PET/CT in our center between January, 2005 and January, 2016. The location, morphologies and metabolic features of the lesions were analyzed in these patients. RESULTS: In the 23 PIL patients, diffusive large B cell lymphoma (DLBCL) and enteropathy- associated T cell lymphoma (EATL) were the primary histopathological types, accounting for 47...
August 20, 2016: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
Annalisa Roberti, Maria Pamela Dobay, Bettina Bisig, David Vallois, Cloé Boéchat, Evripidis Lanitis, Brigitte Bouchindhomme, Marie-Cécile Parrens, Céline Bossard, Leticia Quintanilla-Martinez, Edoardo Missiaglia, Philippe Gaulard, Laurence de Leval
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 tumour-normal tissue pairs. The tumour 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...
2016: Nature Communications
Jeremy Woodward
Intestinal inflammation and symptoms of celiac disease (CD) usually respond well to gluten withdrawal, but rare cases are refractory to diet. Two types of refractory CD are discriminated on the basis of the presence or absence of an atypical population of mucosal lymphocytes that may progress to enteropathy-associated T-cell lymphoma. Challenges remain in the secure diagnosis of both types of refractory disease, and evidence on which to base treatment recommendations is flawed by the small numbers of reported patients and the use of different diagnostic strategies...
2016: Clinical and Experimental Gastroenterology
Joo Y Song, Liping Song, Alex F Herrera, Girish Venkataraman, Joyce L Murata-Collins, Victoria H Bedell, Yuan Yuan Chen, Young S Kim, Reda Tadros, Bharat N Nathwani, Dennis D Weisenburger, Andrew L Feldman
Cyclin D1 is an important regulator of the cell cycle and overexpression of this protein by immunohistochemistry is characteristically seen in mantle cell lymphoma and other B-cell neoplasms. However, little is known about the expression of this protein in T-cell lymphomas. Cyclin-dependent kinase pathway inhibitors are in development, therefore identifying cyclin D1-positive T-cell lymphomas may provide a therapeutic target in a disease where novel treatments are urgently needed. We collected 200 peripheral T-cell lymphomas from three institutions including the following types of cases: 34 anaplastic large cell lymphoma, ALK+, 44 anaplastic large cell lymphoma, ALK negative, 68 peripheral T-cell lymphomas, not otherwise specified, 24 angioimmunoblastic T-cell lymphomas, 7 extranodal NK/T-cell lymphomas, 4 enteropathy associated T-cell lymphomas, 3 hepatosplenic T-cell lymphomas, 12 cutaneous T-cell lymphomas, and 4 large granular lymphocytic leukemias...
July 29, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Hideki Ishibashi, Satoshi Nimura, Yoshiyuki Kayashima, Yasushi Takamatsu, Kunihiko Aoyagi, Naohiko Harada, Masanori Kadowaki, Takihiko Kamio, Shotaro Sakisaka, Morishige Takeshita
BACKGROUND: In East Asia, monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), previously known as type II enteropathy-associated T-cell lymphoma (EATL), occurs more frequently than type I EATL, and coeliac disease is rare. CASE PRESENTATION: Here we present four cases of MEITL in Japanese patients, including the endoscopic and pathological findings of their duodenal and colorectal lesions. Tumor specimens obtained from duodenal, intestinal, and colorectal biopsies in all four patients showed a diffuse intramucosal infiltration of small to/or medium-sized lymphoma cells and numerous atypical intraepithelial lymphocytes (IELs)...
2016: Diagnostic Pathology
C C Liong, S Ravindran, G Gnana Kumar, E F Chin, P S Koh, W K Chan
Chronic diarrhoea in tropical countries may be due to a myriad of causes from infective to non-infective. This case report illustrates the challenges faced in the investigation of a middle-age Chinese gentleman who presented with chronic diarrhoea and weight loss. The diagnosis of type II enteropathy-associated T-cell lymphoma (EATL) was finally made. The diagnosis of EATL was least suspected as the condition is almost unheard of in this part of the world. The epidemiology, presentation, diagnosis, management and prognosis of this rare condition are discussed...
April 2016: Medical Journal of Malaysia
Fang Yu, Jing Xu, Zhinong Jiang
No abstract text is available yet for this article.
January 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Fabienne Charbit-Henrion, Anja Koren Jeverica, Bernadette Bègue, Gasper Markelj, Marianna Parlato, Simona Lucija Avčin, Isabelle Callebaut, Marc Bras, Mélanie Parisot, Janez Jazbec, Matjaz Homan, Alojz Ihan, Frédéric Rieux-Laucat, Marie-Claude Stolzenberg, Frank M Ruemmele, Tadej Avčin, Nadine Cerf-Bensussan
OBJECTIVE: Early onset inflammatory bowel diseases can result from a wide spectrum of rare Mendelian disorders. Early molecular diagnosis is crucial to define treatment and improve life expectancy. Herein we aimed at defining the mechanism of an IPEX-like disease (Immunodeficiency-Polyendrocrinopathy and Enteropathy-X-linked) combined with a severe immunodeficiency in two siblings born from distantly related parents. METHODS: Whole exome sequencing was performed on blood-extracted genomic DNA from the two affected children and their parents on the genomic platform of Institut IMAGINE...
June 2, 2016: Journal of Pediatric Gastroenterology and Nutrition
Abdoul Aziz Garba, Harissou Adamou, Ibrahim Amadou Magagi, Souleymane Brah, Oumarou Habou
Enteropathy associated T-cell lymphoma (EATL) is a rare complication of celiac disease (CD). We report a case of EATL associated with CD revealed by acute intestinal obstruction. A North African woman of 38 years old with a history of infertility and chronic abdominal pain was admitted in emergency with acute intestinal obstruction. During the surgery, we found a tumor on the small intestine with mesenteric lymphadenopathy. Histology and immunohistochemistry of the specimen objectified a digestive T lymphoma CD3+ and immunological assessment of celiac disease was positive...
2016: Pan African Medical Journal
Christopher W Soon, Ilan R Kirsch, Andrew J Connolly, Bernice Y Kwong, Jinah Kim
The presence of eosinophils within the neutrophilic infiltrates of acute febrile neutrophilic dermatosis (Sweet syndrome) is documented in the literature. Here, the authors describe a case of eosinophil-rich acute febrile neutrophilic dermatosis in the setting of new onset enteropathy-associated T-cell lymphoma (EATL), type 1. Histopathologic evaluation of the skin biopsies demonstrated a mixed superficial perivascular and inflammatory infiltrate composed of neutrophils, lymphocytes, and abundant eosinophils...
September 2016: American Journal of Dermatopathology
Vittorio Perfetti, Fausto Baldanti, Marco Vincenzo Lenti, Alessandro Vanoli, Federico Biagi, Marta Gatti, Roberta Riboni, Elena Dallera, Marco Paulli, Paolo Pedrazzoli, Gino Roberto Corazza
Refractory celiac disease is characterized by mucosal damage in patients with celiac disease despite a gluten-free diet. Little is known about the mechanisms that cause persistent intestinal inflammation in these patients. We performed a case-control study of 17 consecutive patients diagnosed with refractory celiac disease from 2001 through 2014 (median age, 51 y; 10 women) and 24 patients with uncomplicated celiac disease (controls) to determine whether refractory disease is associated with infection by lymphotropic oncogenic viruses...
August 2016: Clinical Gastroenterology and Hepatology
Federica Branchi, Martina Locatelli, Carolina Tomba, Dario Conte, Francesca Ferretti, Luca Elli
Celiac disease is the most common autoimmune enteropathy in Western countries, and is usually associated with a good response to the gluten free diet and an excellent prognosis. However, a minority of patients develop complications of the disease, such as refractory celiac disease, ulcerative jejunoileitis and neoplastic complications such as adenocarcinoma of the small bowel and enteropathy associated T cell lymphoma. Neoplastic complications described in association with celiac disease have a high mortality rate, due to their aggressive behavior and to the usual advanced stage at the time of diagnosis...
June 2016: Digestive and Liver Disease
Tsutomu Tanaka, Hideko Yamamoto, Ahmed Ali Elsayed, Akira Satou, Naoko Asano, Kei Kohno, Tomohiro Kinoshita, Yasumasa Niwa, Hidemi Goto, Shigeo Nakamura, Seiichi Kato
The differential diagnosis of primary gastrointestinal EBV T-cell lymphoma (GITCL) includes enteropathy-associated T-cell lymphoma (EATL), peripheral T-cell lymphoma, not otherwise specified, adult T-cell leukemia/lymphoma, and anaplastic large cell lymphoma. Type II EATL is considered to be a tumor of intraepithelial lymphocytes. However, the evaluation of intraepithelial lymphocytosis by biopsy specimens is challenging, which poses a diagnostic problem between the EATL and peripheral T-cell lymphoma, not otherwise specified...
June 2016: American Journal of Surgical Pathology
Jiu-Cong Zhang, Yong Wang, Xiu-Feng Wang, Fang-Xin Zhang
Enteropathy-associated T-cell lymphoma (EATL) is a rare gastrointestinal non-Hodgkin's lymphoma, originating from intraepithelial T-lymphocyte, which is specifically associated with celiac disease. EATL most commonly presents in the sixth and seventh decades of life. We report a unique case of type I EATL in the colon with liver metastasis, which was presented with nonspecific radiological findings and at a very young age (29 years old) compared with previously published data. We suggest that EATL should be regarded as part of differential diagnosis in any patient presenting with abdominal pain, diarrhea, weight loss, and malabsorption because delay in treatment can result in an irreversible clinical outcome...
2016: OncoTargets and Therapy
Yunxin Chen, Soo-Yong Tan, Bengt Fredrik Petersson, Yiu Ming Khor, Sathish Kumar Gopalakrishnan, Daryl Tan
Monomorphic epitheliotropic intestinal T-cell lymphomas (MEITL), formerly Type II enteropathy-associated T-cell lymphomas (EATL), are rare peripheral T-cell lymphomas. They are associated with poor survival outcomes, in part because of their late diagnosis. Although MEITLs may be reliably diagnosed based on histological and immunophenotypic findings, overlaps with other NK/T and T-cell lymphomas may confound the diagnosis. The distinctive high-level nuclear staining of the novel marker Megakaryocyte-associated tyrosine kinase (MATK) in MEITLs is an invaluable tool in distinguishing MEITL from classical EATL and other NK/T or T-cell lymphomas...
March 7, 2016: Hematological Oncology
Megan M Morsheimer, Jack Rychik, Lisa Forbes, Kathryn Dodds, David J Goldberg, Kathleen Sullivan, Jennifer R Heimall
BACKGROUND: Congenital cardiac anomalies are associated with immunologic perturbations. Surgical thymectomy, thoracic duct manipulation, and protein- losing enteropathy (PLE), a condition related to stressed Fontan hemodynamics, presumably contribute to low peripheral absolute lymphocyte counts (ALCs) and quantitative immunoglobulins. Clinical significance of lymphopenia and hypogammaglobulinemia in single-ventricle survivors requires additional study. OBJECTIVE: Although immunologic laboratory anomalies are common in this population, we hypothesize that clinically significant immunodeficiency requiring intervention is rarely required...
May 2016: Journal of Allergy and Clinical Immunology in Practice
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