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Showing results for tags 'enteropathy-associated'.
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Celiac.com 02/19/2024 - A recent study presented at the 2023 annual meeting of the American College of Gastroenterology has raised concerns about the increasing incidence of enteropathy-associated T-cell lymphoma (EATL) – a rare and aggressive form of T-cell, non-Hodgkin lymphoma. This alarming trend has prompted researchers to explore the possible connection between EATL and celiac disease, shedding light on the risks faced by individuals with this autoimmune condition. Lead investigator Dr. Isabel Hujoel, Clinic Director of the Celiac Disease Center at UW Medical Center, Seattle, highlighted the strong association between EATL and celiac disease. While EATL is rare, most cases are observed in patients with celiac disease, suggesting a potential link between the two conditions. The study, utilizing data from the Surveillance, Epidemiology, and End Results (SEER) program database, identified 463 cases of EATL between 2000 and 2020, with an age-adjusted incidence rate of 0.014 per 100,000 people. Alarmingly, the incidence of EATL increased by 2.58% annually over this 20-year period. Despite advancements in medical treatment, the prognosis for EATL remains poor, with a median survival of approximately six months. Findings from the study revealed that most cases were treated with a combination of surgery and chemotherapy. However, survival outcomes did not improve over the study period, underscoring the urgent need for more effective treatment strategies. Dr. Sophia Dar, a gastroenterology fellow at Southern Illinois University School of Medicine, emphasized the importance of early detection and treatment. While chemotherapy showed promising results, the overall mortality rate remained high, highlighting the challenges in managing this aggressive cancer. Researchers emphasized the need for further investigation into the factors contributing to the high mortality rate associated with EATL. Understanding these factors could pave the way for more efficient treatment plans and improved outcomes for patients. Debra Silberg, MD, PhD, Chief Scientific Officer of the nonprofit Beyond Celiac, emphasized the rarity of EATL and the need for targeted screening. Screening for EATL should be considered in cases of refractory celiac disease or when there is suspicion of complications related to celiac disease. The rise in cases of EATL serves as a sobering reminder of the potential complications associated with celiac disease. Heightened awareness, early detection, and improved treatment options are crucial in addressing this rare but deadly cancer among individuals with celiac disease. Read more at gastroendonews.com
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Celiac.com 10/25/2016 - Some potentially big news for people who suffer from enteropathy-associated T-cell lymphoma (EATL), an intestinal tumor that targets intraepithelial T lymphocytes. EATL may be preceded by refractory celiac disease (RCD), which resists treatment with gluten-free diet. In almost all cases, a range of the tumor cells express CD30. RCD occurs in two types based on the absence (type I) or presence (type II) of abnormal intraepithelial lymphocytes (IEL) showing down-regulation of CD8 and often TCR gamma genes rearrangement. RCD II is now considered as a small cell intraepithelial T-cell lymphoma that could be an intermediate stage between celiac disease and EATL. A team of researchers recently set out to establish the pattern of CD30 expression in EATL, which help to improve therapies with the use of anti-CD30 monoclonal antibody like brentuximab vedotin (BV). The research team included David Sibon, Georgia Malamut, Virginie Verkarre, Coralie Derrieux, Isabelle Radford, Bertrand Meresse, Elizabeth Macintyre, Christophe Cellier, Nadine Cerf-Bensussan, Nicole Brousse, and Olivier Hermine. For their study, the team enrolled consecutive adult patients diagnosed with EATL between 2007 and 2013 in Necker University Hospital and Georges Pompidou European Hospital. The team confirmed celiac diagnosis using histopathologic and immunohistochemical review. For the purpose of the present study, two expert hematopathologists reviewed all EATL and extended the phenotypic analysis to reclassify them according to the 2008 WHO classification. The team used a panel of antibodies directed against CD20, CD3, CD4, CD5, CD8, CD56, granzyme B and ALK1. They also performed CD30 staining with Monoclonal Mouse Anti-Human CD30, Clone Ber-H2 (Dako). As a control group, they used consecutive RCDI and RCDII cases with complete phenotype and clonality analyses diagnosed in the same period. They based celiac disease diagnosis on HLA-DQ2/8 typing, detection of celiac specific antibodies, and of villous atrophy with increased counts of IEL on normal diet. They classified patients RCDI or II, depending on their clinical and histological response to a gluten-free diet, and the presence of abnormal IEL. In all 25 cases of EATL, large tumor cells strongly expressed CD30. In RCDI and II, CD30 was negative in most cases (∼90%), and was rarely expressed by dispersed atypical lymphocytes, whether in IEL or in lamina propria. TCR gamma genes were clonally rearranged in 11/14 (79%) EATL, 3/25 (12%) RCDI and 18/20 (90%) RCDII. Based on their findings, the team began a pilot study in 2012. The pilot study combines BV with chemotherapy followed by autologous stem-cell transplantation (ASCT) as frontline treatment for EATL. The team has treated five patients to date. For chemotherapy, the the first two patients received IVE/MTX. After presenting their initial results at ASH 2012 Annual Meeting, the team replaced IVE/MTX with CHP regimen, and treated 3 more patients. Both treatments were well tolerated, and all 5 patients reached clinical remission, and underwent ASCT. EATL type I strongly expresses CD30. Promising results from combining BV with CHP led the team to plan a phase 2 study of BV and CHP, followed by ASCT, as frontline treatment of EATL. Source: Blood 2013 122:4252.
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Celiac.com 08/24/2015 - A new study reveals that U.S. Asians experience higher rates of deadlier cases of Enteropathy-associated T-cell lymphoma EATL. Enteropathy-associated T-cell lymphoma is a rare primary intestinal non-Hodgkin lymphoma (NHL) strongly associated with celiac disease. It is an aggressive disease with a median survival of approximately 10 months (Ferreri et al, 2011). Previous studies suggest that EATL may be more common in Europe and among Whites, among whom celiac disease is prevalent (Delabie et al, 2011; Ferreri et al, 2011). However, a second type of EATL (Type II) not associated with celiac disease is increasingly reported in Asia (Lee et al, 2005; Sun et al, 2011; Tan et al, 2013). To date, there have been no comparative epidemiological study in a racially diverse large population. A team of researchers recently set out to conduct such a study. The research team included Pawan K. Karanam, Mohammed Al-Hamadani, and Ronald S. Go. They are variously associated with the Departments of Medical Education and Medical Research at the Gundersen Medical Foundation in La Crosse, USA, and with the Division of Hematology at the Mayo Clinic, and the Mayo Clinic's Robert D, and Patricia E. Kern Center for the Science of Health Care Delivery, Rochester, MN, USA. The team turned to the two largest public cancer databases in the US: the Surveillance, Epidemiology, and End Results (SEER) database (http://www.seer.cancer.gov); and the National Cancer Data Base (NCDB; http://www.facs.org/quality-programs/cancer/ncdb). Using these databases, the research team was able to find and compare the cases of EATL by race. They were also able to describe the clinical features and overall survival (OS) for these cases. The team's study included all patients with an EATL diagnosis according to International Classification of Diseases for Oncology (ICD-O: 9717). The team used SEER-18 registries from 2000 to 2011 to calculate incidence. To describe clinical outcomes, they used the NCDB NHL-PUF with patients diagnosed between 1998 and 2012 for clinical characteristics and those diagnosed between 1998 and 2006 for OS. Because CoC-accredited programs report survival data only once every 5 years, OS analysis was possible only for patients diagnosed between 1998 and 2006. From the data, the team calculated the incidence rate (case/1 000 000), age-adjusted to the 2000 standard US population, according to race (White, Black, Asian/Pacific Islander, American Indian/Alaska native) using seer*stat software version 8.1.5 (National Cancer Institute, Bethesda, MD, USA) and performed risk ratio comparisons using Poisson regression. They analyzed OS using the Kaplan–Meier method and used log-rank tests to compare survival distributions between race cohorts. The prognostic effect of pertinent clinical variables were studied using multivariate Cox proportional hazards models. They found that, for the years 2000–2010, the overall age-adjusted incidence rate of EATL in the US was 0·111 per 1,000,000. Asians/Pacific Islanders had a higher incidence rate (0·236) compared with other races [White (0·101), Black (0·107), American Indian/Alaska native (0·128)]. The risk ratio of Asians/Pacific Islanders compared with non–Asians/Pacific Islanders was 2·32 [95% confidence interval (CI) 1·39–3·69; P = 0·002]. The incidences for Asians and Pacific Islanders were combined in seer*stat, therefore we could not provide separate incidences for Asians and Pacific Islanders. All tests of statistical significance were two-sided and P < 0·05 was considered significant. Source: British Journal of Haematology, Vol. 170 Issue 3. DOI: 10.1111/bjh.13555
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Celiac.com 08/01/2008 - One of the particularly aggressive and deadly types of cancer associated with celiac disease in adults is known as Enteropathy-associated T-cell lymphoma (EATL), which is a T-cell non-Hodgkin lymphoma that develops in the small bowel. So, if you haven’t heard of EATLs, you should know that while current estimates indicate that even though EATLs are rare overall, they are one of the most common causes of death in people with celiac disease. One problem with studying EATLs is that the best statistical information regarding its prevalence is still based on estimates. Until recently, there had been no study made to determine the rate at which EATLs occur in the general population. A team of doctors based in the Netherlands recently set out to conduct such an assessment using the Dutch national network and patient registry of cyto- and histopathology reports (PALGA). The research team included Wieke H. M. Verbeek, Jolanda M. W. Van de Water, Abdulbaqi al-Toma, Joost J. Oudejans, Chris J. J. Mulder & Veerle M. H. Coupé. The team looked at all T-cell lymphomas found from January 2000 to December 2006 that originated in the small bowel, and they computed some basic average rates of EATL occurrence for the Netherlands and worldwide, along with occurrence rates by gender and age. The team also factored in the location of the lymphoma, Marsh categorization for celiac disease, and the means by which the patients’ lymphomas were detected. In people with celiac disease, eating wheat causes the wheat protein to trigger an adverse immune reaction that leads to inflammation of the intestinal lining, which can eventually cause the cells in the inflamed region to become cancerous. Even though celiac disease occurs twice as often in women as in men, men are far more likely to develop EATLs. Out of every 10 people who develop EATLs, only 2 to 5 of them have any obvious symptoms. Also, these statistics apply to untreated celiacs, and those diagnosed as adults, while people diagnosed as children and following a gluten-free diet have about the same rates of EATL as the general population. Adults with untreated celiac disease are nearly 70 times more likely to die from lymphoma than people without celiac disease. Again, since more and more people are being diagnosed with celiac disease as adults, it’s important to get the clearest possible picture of the associated risks, especially when they are as serious as EATLs. The team also noted that most EATLs seemed to be centered in theproximal small intestine, and that diagnosis was generally madesurgically. The team looked at 116 incidents of EATL and found a rate in the general Dutch population of .10/100,000. This is about double the estimated western rate of about .05/100,000. For those over 50 years of age, the Dutch rate of EATL increased by a factor of 10 to 2.08/100,000, while over 60, the Dutch rate was 2.92/100,000. Still, in addition to afflicting almost only those with celiac disease, EATL seems to afflict mostly men. For those over 50, EATL rates were .09/100,000 for women, but nearly 3 times that, 2.95/100,000 for men. One interesting part of the study was the acknowledgment by the doctors that increased cancer rates in celiacs have not been judged “sufficiently large” to warrant screening the general population that way some countries do. Instead, the doctors have adopted a strategy of checking patients with EATL for celiac disease. By their own admission, most patients with EATL have already been diagnosed with celiac disease. In any case, if you have a particularly deadly type of cancer it would seem a little late to test you for celiac disease. We at Celiac.com propose that a better strategy would be to test those with celiac disease for EATLs (and screen the general population for gluten intolerance). This study drives home the importance of diagnosing and treating celiac disease as early as possible, and also reinforces the importance of faithfully following a gluten-free diet and getting regular follow-up biopsies and screening that would reveal an EATL. Article citation: Scandinavian Journal of Gastroenterology Published on July 11, 2008 DOI: 10.1080/00365520802240222
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Celiac.com 05/19/2010 - Enteropathy-associated T cell lymphoma is a serious complication of celiac disease, and a major cause of mortality in untreated celiac disease. One possible trigger for Enteropathy-associated T cell lymphoma development is chronic exposure of intraepithelial lymphocytes (IELs) to strong anti-apoptotic signals, that is, signals that interfere in the normal mortality of the IEL cells. These signals are triggered by IL-15, a cytokine that is over-expressed in the enterocytes of people with celiac disease. However, researchers have not yet fully mapped the signaling pathway by which IL-15 transmits these anti-apoptotic signals. Researchers consider type II refractory celiac disease (RCDII) to be a middle step between celiac disease and enteropathy-associated T cell lymphoma. Eliminating abnormal IELs at the RCDII stage would likely block EATL development. So far, though, scientists have not found successful immunosuppressive and/or chemotherapeutic approaches able to accomplish this, and RCDII outcomes remain very poor. A team of researchers recently set out to map the IL-15–driven survival pathway in human IELs, and to determine whether IL-15 triggered pathway in human intraepithelial lymphocytes represents a possible new target in type II refractory celiac disease and enteropathy-associated T cell lymphoma. The research team was made up of Georgia Malamut, Raja El Machhour, Nicolas Montcuquet, Séverine Martin-Lannerée, Isabelle Dusanter-Fourt, Virginie Verkarre, Jean-Jacques Mention, Gabriel Rahmi, Hiroshi Kiyono, Eric A. Butz, Nicole Brousse, Christophe Cellier, Nadine Cerf-Bensussan, and Bertrand Meresse. The are variously affiliated with INSERM U989, the Université Paris Descartes, Faculté de Médecine René Descartes, the Department of Gastroenterology, AP-HP, Hôpital Européen Georges Pompidou, the Institut Cochin, Université Paris Descartes, CNRS (UMR 8104), INSERM U1016, the Department of Pathology, AP-HP, of the Hôpital Necker-Enfants Malades in Paris, with the Division of Mucosal Immunology, Department of Microbiology and Immunology at the Institute of Medical Science at the University of Tokyo in Japan, and the Inflammation Department of AMGEN Inc., in Seattle, Washington, USA. Their current findings reveal that the survival signals IL-15 directs to freshly isolated human IELs, and to human IEL cell lines derived from celiac disease patients with type II refractory celiac disease, depend on anti-apoptotic factors Bcl-2 and/or Bcl-xL. The signals require IL-15Rβ, Jak3, and STAT5 for proper function, but functioned independently of PI3K, ERK, and STAT3. In support of these findings, the team recorded elevated levels of Bcl-xL, phospho-Jak3, and phospho-STAT5 in IELs from patients with active celiac disease and RCDII. Moreover, by incubating patient duodenal biopsies with a fully humanized human IL-15–specific Ab, the team effectively blocked Jak3 and STAT5 phosphorylation. Also, treatment with IL-15–specific Ab caused IEL cell mortality, and wiped out the massive IEL build-up in mice over-expressing human IL-15 in their gut epithelium. The study marks the first successful mapping of the IL-15–driven survival pathway in human IELs, and demonstrates that IL-15 and its downstream effectors are meaningful therapeutic targets in RCDII. These findings will likely help to pave the way for the development of successful immunosuppressive and/or chemotherapeutic treatments that destroy abnormal IELs at the RCDII stage and help to block EATL development, improving outcomes for RCDII patients. Source: Journal of Clinical Investigation doi:10.1172/JCI41344
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Celiac.com 05/18/2010 - A research team recently concluded a clinicopathologic and array comparative genomic hybridization study on enteropathy-associated T-cell lymphoma. The team included Young Hyeh Ko MD, PhD; Sivasundaram Karnan; Kyeong Mee Kim MD, PhD; Cheol Keun Park MD, PhD; Eun Suk Kang MD, PhD; Young Ho Kim MD, PhD; Won Ki Kang MD, PhD; Seok Jin Kim MD, PhD; Won Seog Kim MD, PhD; Woo Yong Lee MD, PhD; Ho Kyung Chune; Masao Seto MD, PhD. The are associated variously with the Department of Pathology, the Department of Laboratory Medicine, the Division of Gastroenterology, Hemato-oncology of Internal Medicine, the Department of General Surgery of Samsung Medical Center at Sungkyunkwan University in Seoul, Korea and the Division of Molecular Medicine of the Aichi Cancer Center Research Institute in Nagoya, Japan. The latest World Health Organization classification system recognizes 2 types of enteropathy-associated T-cell lymphoma. The first, EATL type 1, is strongly associated with celiac disease, and makes up most EATL cases in Western countries. The second, EATL type 2 has no associations with celiac disease. To properly classify enteropathy-associated T-cell lymphoma types in Korea, the team conducted clinicopathologic and immunophenotypic analyses of 8 Koreans with enteropathy-associated T-cell lymphoma, and investigated genomic profile via array comparative genomic hybridization. Five patients presented tumors in the small intestine, while three presented tumors in the colorectum. Two patients carried an HLA DQB1âŽ0302 allele that corresponds to HLA DQ8. None of the patients suffered gluten-sensitive malabsorption syndrome. The team found intraepithelial lymphocytosis in all patients. In seven patients showed small, or small-to-medium, tumor cells. One patient presented with a medium-to-large tumor. Tumor cell immunophenotypes were CD4−CD8+CD56+ in 4 cases, CD4−CD8+CD56− in 1 case, CD4−CD8−CD56+ in 2 cases, and CD4−CD8−CD56− in 1 case. Using array comparative genomic hybridization analysis to spot recurring genomic alterations, the team found chromosome 9q33-q34.1 gain in four of five patients, chromosome 6p21.1-21.31 gain in three of five (60%), chromosome 6p21.1-21.31 gain in three of five (60%), and chromosome 3p12.1-p12.2 and 3q26.31 loss in two out of five (40%). These results show type 2 enteropathy-associated T-cell lymphoma to be the most prevalent type in this geographic region, and that associated genetic changes are similar to those in Western countries. Source: Human Pathology (2010) doi:10.1016/j.humpath.2009.11.020
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Celiac.com 08/17/2008 - One of the important ways doctors distinguish between the two types of refractory celiac disease is by looking at differences in intra-epithelial T lymphocytes (IELs) in intestinal biopsies. People with refractory celiac disease who show normal IELs are said to have refractory celiac disease I, while those with abnormal IELs are said to have refractory celiac disease II. A team of doctors based in the Netherlands recently set out to assess the effectiveness of computed tomography (CT) in diagnosing refractory celiac disease, and enteropathy-associated T-cell lymphoma (EATL). EATL is a generally rare, but particularly aggressive form of bowel cancer that is the leading cause of death in adults with celiac disease. The study team was made up of doctors Maarten Mallant, Muhammed Hadithi, Abdul-Baqi Al-Toma, Matthijs Kater, Maarten Jacobs, Radu Manoliu, Chris Mulder, and Jan Hein van Waesberghe. The team looked at 46 patients with clinically proven celiac disease, refractory celiac disease I, refractory celiac disease II, or EATL including 18 males and twenty-eight females. The first group contained 14 patients with uncomplicated celiac disease and 10 with type I refractory celiac disease. The second group contained 15 patients with type II refractory celiac disease and 7 patients with EATL. 5 patients from group II showed lymphandenopathy, compared to none in the first group. 20 patients from group I showed a higher number of small mesenteric vessels compared to just 11 from group II. This is significant because increased numbers of small mesenteric vessels are associated with an absence of refractory celiac disease II and EATL, while reduced numbers of small mesenteric vessels are associated with a higher rate of refractory celiac disease II and EATL. The team evaluated the two groups within eleven categories: abnormal intestinal fold patterns; bowel wall thickness, excess fluid; intestinal insussuction; ascites; lymphadenopathy; increases in lymph node numbers; mesenteric vascular changes; and spleen size. One other area the doctors found important was in differences in the average thickness of the bowel wall. Group I showed thinner bowel walls compared to group II. In group I, average bowel thickness ranged from 4mm to 11mm, with an average thickness of 7.0mm. In group II, average bowel thickness ranged from 5mm to 15mm, with an average thickness of 10.0mm. So, group II showed about 30% thicker bowel walls than group I. The doctors’ conclusions reaffirmed the need for a biopsy before confirming a diagnosis of celiac disease. Regarding the use of CT, the team found CT unnecessary for cases of uncomplicated celiac disease, but found CT very useful in cases of complicated and pre-cancerous celiac disease. The study team also found that pattern reversal and/or loss of jejunal folds is specific to celiac disease, though they had an admittedly small sample of just 24 of their 46 patients, so their measures are far from definitive. All of this drives home the importance of encouraging early and accurate screening for celiac disease. Ideally, we will get to the point where, like many European countries, we will begin to catch celiac disease before it ever becomes refractory, and before it ever develops into EATL. Until then, stay informed and take an active role in maintaining your own health. World J Gastroenterol 2007; 13(11): 1696-1700
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