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Enteropathy Associated T Cell Lymphoma

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https://www.readbyqxmd.com/read/28533835/-primary-colonic-extranasal-nk-t-cell-lymphoma-about-a-case
#1
Salma Fares, Mouna Lamchahab, Myriem Aniba, Ghizlane Lembarki, Nadia Mousalli, Meriem Regragui, Mehdi Karkouri, Asmaa Quessar
To better understand this cancer, we here report the case of a 43-year old patient diagnosed with localized and isolated primary colonic NK/T-cell lymphoma without associated enteropathy, treated wih 3 cycles of AspaMetDex with a poor response who died during treatment with a clinical picture of acute abdomen. Primary intestinal NK/T-cell lymphoma most commonly affects the young subject with poor prognosis. It is difficult to distinguish between intestinal NK/T-cell lymphoma and inflammatory or infectious intestinal disorders because of its non-specific clinical and endoscopic features...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28507749/splenic-volume-differentiates-complicated-and-non-complicated-celiac-disease
#2
Tom van Gils, Petula Nijeboer, Jan Hein Tm van Waesberghe, Veerle Mh Coupé, Kiki Janssen, Jessy A Zegers, Shaikh A Nurmohamed, Georg Kraal, Sabine Ci Jiskoot, Gerd Bouma, Chris Jj Mulder
BACKGROUND: Studies in small groups of patients indicated that splenic volume (SV) may be decreased in patients with celiac disease (CD), refractory CD (RCD) type II and enteropathy-associated T-cell lymphoma (EATL). OBJECTIVE: The objective of this article is to evaluate SV in a large cohort of uncomplicated CD, RCD II and EATL patients and healthy controls. METHODS: The retrospective cohort consisted of 77 uncomplicated CD (of whom 39 in remission), 29 RCD II, 24 EATL and 12 patients with both RCD II and EATL...
April 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28424246/enteropathy-associated-t-cell-lymphoma-subtypes-are-characterized-by-loss-of-function-of-setd2
#3
Andrea B Moffitt, Sarah L Ondrejka, Matthew McKinney, Rachel E Rempel, John R Goodlad, Chun Huat Teh, Sirpa Leppa, Susanna Mannisto, Panu E Kovanen, Eric Tse, Rex K H Au-Yeung, Yok-Lam Kwong, Gopesh Srivastava, Javeed Iqbal, Jiayu Yu, Kikkeri Naresh, Diego Villa, Randy D Gascoyne, Jonathan Said, Magdalena B Czader, Amy Chadburn, Kristy L Richards, Deepthi Rajagopalan, Nicholas S Davis, Eileen C Smith, Brooke C Palus, Tiffany J Tzeng, Jane A Healy, Patricia L Lugar, Jyotishka Datta, Cassandra Love, Shawn Levy, David B Dunson, Yuan Zhuang, Eric D Hsi, Sandeep S Dave
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...
May 1, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28344788/lymphoma-development-and-survival-in-refractory-coeliac-disease-type-ii-histological-response-as-prognostic-factor
#4
P Nijeboer, Rlj van Wanrooij, T van Gils, N J Wierdsma, G J Tack, B I Witte, H J Bontkes, O Visser, Cjj Mulder, G Bouma
BACKGROUND: Refractory coeliac disease type II (RCDII) frequently transforms into an enteropathy-associated T-cell lymphoma (EATL) and therefore requires intensive treatment. Current evaluated treatment strategies for RCDII include cladribine (2-CdA) and autologous stem cell transplantation (auSCT). OBJECTIVE: The purpose of this study was to evaluate long-term survival and define clear prognostic criteria for EATL development comparing two treatment strategies...
March 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28323276/open-capsule-budesonide-for-refractory-celiac-disease
#5
Saurabh S Mukewar, Ayush Sharma, Alberto Rubio-Tapia, Tsung-Teh Wu, Bana Jabri, Joseph A Murray
OBJECTIVES: Refractory celiac disease (RCD) is a rare condition often associated with poor prognosis. Various immunosuppressive medications (IMs) have been used with modest success. We describe outcomes in patients treated with open-capsule budesonide (OB), including those for whom IM treatment failed. METHODS: We identified RCD patients treated with OB at Mayo Clinic, Rochester, Minnesota from 2003 to 2015. Demographic, serologic, and clinical variables were analyzed...
March 21, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28316767/type-ii-enteropathy-associated-t-cell-lymphoma-a-rare-report-from-iran
#6
Neda Nozari
Type II enteropathy-associated T-cell lymphoma (EATL) is a rare disease that is reported from Asia, especially from Taiwan. In this paper, we present a patient from Iran (Arab) with Type II EATL whose disease was not associated with celiac disease or Epstein-Barr virus (EBV) infection. The patient died due to intestinal perforation during the first chemotherapy. Type II EATL is a gastrointestinal lymphoma with poor prognosis and a high affinity for intestinal perforation. Early detection of EATL II is essential for chemotherapy prior to the onset of malnutrition symptoms...
January 2017: Middle East Journal of Digestive Diseases
https://www.readbyqxmd.com/read/28222648/autologous-stem-cell-transplantation-as-frontline-strategy-for-peripheral-t-cell-lymphoma-a-single-centre-experience
#7
Xiao Han, Wei Zhang, Daobin Zhou, Jing Ruan, Minghui Duan, Tienan Zhu, Jian Li, Huacong Cai, Xinxin Cao, Mingqi Ouyang
Objective To determine the efficacy and prognosis of autologous hematopoietic stem cell transplantation (ASCT) as frontline treatment for peripheral T cell lymphoma (PTCL). Methods Clinical data from 46 PTCL patients who achieved complete (CR) or partial remission (PR) after ASCT from October 1996 to July 2014 were analysed retrospectively. Results Median patient age was 32 (range: 15-68) years. Disease types included PTCL, unspecified type, in 23 patients, anaplastic large cell lymphoma in eight, angioimmunoblastic lymphoma in eight, extranodal NK/T-cell lymphoma in five, and hepatosplenic T-cell lymphoma and enteropathy associated T-cell lymphoma in one each...
February 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28188172/t-cell-repertoires-in-refractory-coeliac-disease
#8
Julia Ritter, Karin Zimmermann, Korinna Jöhrens, Stefanie Mende, Anke Seegebarth, Britta Siegmund, Steffen Hennig, Kremena Todorova, Andreas Rosenwald, Severin Daum, Michael Hummel, Michael Schumann
OBJECTIVE: Refractory coeliac disease (RCD) is a potentially hazardous complication of coeliac disease (CD). In contrast to RCD type I, RCD type II is a precursor entity of enteropathy-associated T-cell lymphoma (EATL), which is associated with clonally expanding T-cells that are also found in the sequentially developing EATL. Using high-throughput sequencing (HTS), we aimed to establish the small-intestinal T-cell repertoire (TCR) in CD and RCD to unravel the role of distinct T-cell clonotypes in RCD pathogenesis...
February 10, 2017: Gut
https://www.readbyqxmd.com/read/28154909/beyond-moulage-sign-and-ttg-levels-the-role-of-cross-sectional-imaging-in-celiac-sprue
#9
Shannon P Sheedy, John M Barlow, Joel G Fletcher, Thomas C Smyrk, Francis J Scholz, Don C Codipilly, Badr F Al Bawardy, Jeff L Fidler
Celiac disease is an autoimmune disorder that causes inflammation and destruction in the small intestine of genetically susceptible individuals following ingestion of gluten. Awareness of the disease has increased; however, it remains a challenge to diagnose. This review summarizes the intestinal and extraintestinal cross-sectional imaging findings of celiac disease. Small intestine fold abnormalities are the most specific imaging findings for celiac disease, whereas most other imaging findings reflect a more generalized pattern seen with malabsorptive processes...
February 2, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28144091/clinicopathological-profile-of-gastrointestinal-lymphomas-in-kashmir
#10
Mehnaaz Sultan Khuroo, Summyia Farooq Khwaja, Ajaz Rather, Zhahid Hassan, Ruby Reshi, Naira Sultan Khuroo
BACKGROUND: The histological categorization of lymphoma has been a source of controversy for many years for both clinicians and pathologists. Clinicopathologic information of gastrointestinal lymphomas in Indian subcontinent is lacking. We studied histopathological spectrum of Primary Gastrointestinal Lymphomas (PGIL) and attempted to classify the G.I. lymphomas based on the recent WHO classification in to major histological types and immunological categories. MATERIAL AND METHODS: This study was done to evaluate the clinicopathological pattern of 100 cases with a histopathological diagnosis of primary gastrointestinal lymphoma at a tertiary care hospital...
October 2016: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28125074/outcome-of-referrals-for-non-responsive-celiac-disease-in-a-tertiary-center-low-incidence-of-refractory-celiac-disease-in-the-netherlands
#11
R L J van Wanrooij, G Bouma, H J Bontkes, A Neefjes-Borst, N C van Grieken, B M E von Blomberg, C J J Mulder
OBJECTIVES: Refractory celiac disease (RCD) is a severe cause of non-responsive celiac disease (CD) due to its association with the enteropathy associated T-cell lymphoma (EATL). Conflicting data exist on the prevalence and the clinical manifestations of RCD type I (RCD I) and type II (RCD II). The aim of the current study was to provide insight in the incidence of RCD and in the distinction with other causes of non-responsive CD. METHODS: A total of 106 CD patients were referred to our tertiary referral center between January 2006 and December 2011 for evaluation of non-responsive CD...
January 26, 2017: Clinical and Translational Gastroenterology
https://www.readbyqxmd.com/read/28078381/celiac-disease-a-clinical-review
#12
Badr Al-Bawardy, D Chamil Codipilly, Alberto Rubio-Tapia, David H Bruining, Stephanie L Hansel, Joseph A Murray
Celiac disease (CD) is an immune-mediated inflammatory enteropathy triggered by gluten exposure in genetically susceptible individuals. It has a high prevalence approaching 1% of the US population. A high index of suspicion is warranted to diagnose CD as frequently patients present with extraintestinal or atypical manifestations. CD is diagnosed by a combination of serum serologies and duodenal biopsies. The majority of patients will respond to a lifelong gluten-free diet which is the cornerstone of therapy...
January 12, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28011041/establishment-of-a-patient-derived-xenograft-of-canine-enteropathy-associated-t-cell-lymphoma-large-cell-type
#13
K S Im, J H Kim, A J Graef, I Cornax, D M Seelig, M G O'Sullivan, R C Kovi, J F Modiano
The pathogenesis of canine T-cell lymphoma remains incompletely understood, partly because there are no well-established in-vivo models to study these malignancies. For this study, we generated a patient-derived tumour xenograft (PDTX) from a 10-year-old neutered male golden retriever dog with enteropathy-associated intestinal T-cell lymphoma, large cell type. One of two female, 15-week-old beige/nude/XID mice developed a visible tumour 7 weeks after sections of tumour material from the spleen were surgically implanted...
January 2017: Journal of Comparative Pathology
https://www.readbyqxmd.com/read/27910801/epidemiologic-and-therapeutic-aspects-of-refractory-coeliac-disease-a-systematic-review
#14
REVIEW
Sara Anna Rowinski, Erik Christensen
INTRODUCTION: Refractory coeliac disease (RCD) is a rare and severe malabsorptive disease. The condition has two subtypes: RCDI and RCDII. Different treatments have been tested: and because RCD has a poor prognosis due to progress to enteropathy-associated T-cell lymphoma, the aim was to review the epidemiologic aspects and the therapeutic options for RCD. METHODS: A systematic literature search was performed in 18 databases, and 122 records were identified. Incidence, prevalence, treatment methods and their efficacy were evaluated...
December 2016: Danish Medical Journal
https://www.readbyqxmd.com/read/27900603/enteropathy-associated-t-cell-lymphoma
#15
REVIEW
Sarah Ondrejka, Deepa Jagadeesh
Enteropathy-associated T-cell lymphoma is a rare neoplasm with uniformly aggressive features that arises from intestinal T-cells. There is strong evidence supporting its association as a dire complication of celiac disease. The clinical presentation can vary from malabsorption and abdominal pain to an acute abdominal emergency. Originally, it was divided into types I and II in World Health Organization (WHO) classification schemes, reflective of epidemiology and differences in clinicopathologic features. The debate over the degree of separation of the two types is ongoing as new data emerges regarding the pathogenetics...
December 2016: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/27885681/dynamics-of-occurrence-of-refractory-coeliac-disease-and-associated-complications-over-25%C3%A2-years
#16
W Eigner, K Bashir, C Primas, L Kazemi-Shirazi, F Wrba, M Trauner, H Vogelsang
BACKGROUND: Refractory coeliac disease, enteropathy associated T-cell lymphoma and small bowel adenocarcinoma are rare but prognostically important complications in coeliac disease. AIM: To analyse potential changes in occurrence of complicated coeliac disease over the last 25 years. METHODS: One thousand one hundred and thirty eight patients were included and evaluated based on their time of first presentation at the Medical University of Vienna, Austria...
January 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/27862162/erythema-multiforme-like-cutaneous-lesions-in-monomorphic-epitheliotropic-intestinal-t-cell-lymphoma-a-rare-case-report
#17
Kumpol Aiempanakit, Chitiarpa Amatawet, Kanokphorn Chiratikarnwong, Sauvarat Auepemkiate, Kanita Kayasut, Supaporn Suwiwat, Benjawan Apinantriyo
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), also known as Type II enteropathy-associated T-cell lymphoma (EATL), is an aggressive peripheral T-cell lymphoma. EATL generally presents in adults with gastrointestinal symptoms. Skin involvement is very rare, found only in approximately five percent of patients. The authors report a 67-year-old Asian male who presented with chronic diarrhea and developed erythema multiforme-like cutaneous lesions. A skin biopsy revealed extensive pagetoid spread of atypical lymphocytes in the epidermis...
February 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/27803799/approach-to-patients-with-refractory-coeliac-disease
#18
REVIEW
Ikram Nasr, Iman Nasr, Hannah Campling, Paul J Ciclitira
Refractory coeliac disease (RCD) is a recognised complication, albeit very rare, of coeliac disease (CD). This condition is described when individuals with CD continue to experience enteropathy and subsequent or ongoing malabsorption despite strict adherence to a diet devoid of gluten for at least 12 months and when all other causes mimicking this condition are excluded. Depending on the T-cell morphology and T-cell receptor (TCR) clonality at the β/γ loci, RCD can be subdivided into type 1 (normal intra-epithelial lymphocyte morphology, polyclonal TCR population) and type 2 (aberrant IELs with clonal TCR)...
2016: F1000Research
https://www.readbyqxmd.com/read/27772962/genomic-and-immunohistochemical-profiles-of-enteropathy-associated-t-cell-lymphoma-in-japan
#19
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
https://www.readbyqxmd.com/read/27766624/enteropathy-associated-t-cell-lymphoma-presenting-as-cutaneous-deposits
#20
Amy Webster, Philip Crea, Mark W Bamford, Roger Hew, Yvette Griffin, Fiona Miall
No abstract text is available yet for this article.
January 2017: British Journal of Haematology
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