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Found 18 results

  1. 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.
  2. Well well well.... Not surprised. Nor scared. I may have cancer again. I am going to go WHOLE HIPPY NUTS THIS TIME! BooYAA! I'm allergic to pain killers: no surgery for me. Thank GOD! I'm not interested at all in chemo or radiation again. Been there done that. They will want to bone marrow transplant, I will not tolerate pain killers nor that transplant without painkillers. lol I am however FAR more aware of what to research, how much to supplement, how little and signs to look out for. I DO have a Naturopathic Doctor licensed and NOT a quake to monitor what I choose to do nutrition wise. Welcome, to the FIGHT! DING DING DING> IT'S ON! LOL Cancer shall meet it's worst enemy..... ME! BWAHAHAHHAHA Any-who, cancer is literally our own healthy cells becoming malignant (going astray/doing something they aren't programed to do by DNA). SO really we should care for our unhealthy cells. Treat them with the herbs, vitamins and minerals that are put here on the face of this earth in their best form by God Himself. I believe I will live as long as the good Messiah allows me to and I will be with Him when He wants me to. I'll just be more educated of the remedies available and be more responsible for the temple (my body) that He has given me. I should have never SLACKED on my immune supports when first diagnosed with Celiac. That was the stupidest mistake, I was worried about the money>which is now being spent ANYWAY. Sigh. Time to get back up in the saddle!
  3. 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
  4. I absolutely adore my gastroenterologist. He is very dedicated to my well being. Unfortunately,I feel that at nearly every appointment that we have he always ends on the note of the possibility of lymphoma. He has a lot of diagnostic appointments for blood work and some barium imaging studies that he has me scheduled for over the next few months that are primarily to r/o cancer. Is this a common fear among celiac patients or should I be concerned? (I am already concerned actually I am just trying to see if this is common practice) Thanks Kortney
  5. Well well well.... Not surprised. Nor scared. I may have cancer again. I am going to go WHOLE HIPPY NUTS THIS TIME! BooYAA! I'm allergic to pain killers: no surgery for me. Thank GOD! I'm not interested at all in chemo or radiation again. Been there done that. They will want to bone marrow transplant, I will not tolerate pain killers nor that transplant without painkillers. lol I am however FAR more aware of what to research, how much to supplement, how little and signs to look out for. I DO have a Naturopathic Doctor licensed and NOT a quake to monitor what I choose to do nutrition wise. Welcome, to the FIGHT! DING DING DING> IT'S ON! LOL Cancer shall meet it's worst enemy..... ME! BWAHAHAHHAHA Any-who, cancer is literally our own healthy cells becoming malignant (going astray/doing something they aren't programed to do by DNA). SO really we should care for our unhealthy cells. Treat them with the herbs, vitamins and minerals that are put here on the face of this earth in their best form by God Himself. I believe I will live as long as the good Messiah allows me to and I will be with Him when He wants me to. I'll just be more educated of the remedies available and be more responsible for the temple (my body) that He has given me. I should have never SLACKED on my immune supports when first diagnosed with Celiac. That was the stupidest mistake, I was worried about the money>which is now being spent ANYWAY. Sigh. Time to get back up in the saddle!
  6. Celiac.com 02/09/2009 - An extensive recent survey of the Swedish cancer registry reveals that people with celiac disease face a 5-fold increased risk of developing non-Hodgkin lymphoma, but that the risk has decreased by more than 50% over the last 40 years. Researchers at the National Cancer Institute (NCI) in Bethesda, Maryland, and Sweden's Karolinska Institute recently undertook a review of more than 60,000 lymphoma cases diagnosed in Sweden between 1965 and 2004. They matched those cases to individual lymphoma-free controls with similar characteristics. Dr. Ying Gao of the NCI and colleagues found 37,869 cases of non-Hodgkin's lymphoma, 8,323 cases of Hodgkin's lymphoma, 13,842 cases of chronic lymphocytic leukemia. The researchers also enrolled 236,408 matched controls and 613,961 first-degree relatives. The team used hospital discharge information to identify people with a history of celiac disease. The data revealed that people with a hospital discharge diagnosis of celiac disease faced a 5.35-fold increased risk of developing non-Hodgkin's lymphoma. The data also showed that risk of Hodgkin's lymphoma was mildly elevated, and thst celiac patients showed no elevated risk of developing chronic lymphocytic leukemia. The data showed that from 1975-1984, patients with celiac disease faced a 13.2-fold greater risk of non-Hodgkin's lymphoma; from 1985-1994, that level fell to a 7.90-fold increased risk, and from 1995-2004 that risk fell again to 3.84-fold increased risk. Siblings of those affected with celiac disease also faced a 2.03-fold greater risk of non-Hodgkin's lymphoma. At present, doctors do not clearly understand the causal link between the two. Earlier studies have indicated that the inflammation common to celiac disease leads drives lymphoma development. According to the research team, the study carries two basic messages: The first is that earlier detection of celiac disease is helping to lower the risk of developing lymphoma over time, so today, fewer people are detected in the late stages, when the risk of lymphoma is much greater. The second message is that people with a family history of celiac disease have a greater chance of developing lymphoma. This family connection was shown to be separate from the personal celiac disease history of the individual. Together, these revelations suggest that shared mechanisms might contribute to both celiac disease and lymphoma. The full report appears in the medical journal Gastroenterology, January 2009.
  7. Celiac.com 04/15/2013 - Enteropathy-associated T cell lymphoma (EATL) is a gut cancer that often ends in death. Currently, doctors have very little idea what factors might help patients survive. The manner in which clinical presentation, pathological features and therapies influence EATL outcome was the subject of a recent study by a team of researchers. The research team included: G. Malamut; O. Chandesris; V. Verkarre; B.Meresse, C. Callens, E. Macintyre, Y. Bouhnik, J.M. Gornet; M. Allez; R. Jian; A. Berger; G. Châtellier; N. Brousse, O. Hermine, N. Cerf-Bensussan, and C. Cellier. They are variously affiliated with the Université Paris Descartes, the Gastroenterology Department of Hôpital Européen Georges Pompidou, APHP, and Inserm U989 in Paris, France. For their study, the team evaluated the medical files of 37 well-documented patients with celiac disease and T-cell lymphoma. They then analyzed lymphoma and intestinal mucosa by histopathology, multiplex PCR and intestinal intraepithelial lymphocytes phenotyping. Using Kaplan-Meier curves with Logrank test and Cox Model they then analyzed patient survival and prognostic factors. They found 15 patients with lymphoma-complicated non-clonal enteropathy, celiac disease, two patients with type I refractory celiac disease, and 20 patients with clonal type II refractory celiac disease. Twenty-five patients underwent surgery with resection of the main tumor mass in 22 cases. Univariate analysis showed that non-clonal celiac disease, serum albumin levels under 21.6g/L at diagnosis, chemotherapy and surgical resection predicted good survival (p=0.0007, p Multivariate analysis showed that serum albumin level>21.6g/L, chemotherapy and reductive surgery were all significantly associated with increased survival rates (p The results reinforce the value of assessing celiac disease type in patients with T-cell lymphoma, and suggest that a combination of nutritional, chemotherapy and reductive surgery may improve survival rates in cases of EATL. Source: Dig Liver Dis. 2013 Jan 9. pii: S1590-8658(12)00438-0. doi: 10.1016/j.dld.2012.12.001.
  8. 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
  9. Celiac.com 01/14/2009 - For decades now, doctors have known that people with celiac disease face a significantly greater risk of developing non-Hodgkin's lymphoma (NHL), though that risk has steadily declined over the last 40 years. Recently though, a team of doctors at the National Cancer Institute in Bethesda, Md., led by Ying Gao, M.D., has discovered that siblings of celiac patients also face an increased risk of developing NHL. Results of the study appeared in the January issue of Gastroenterology. The research team conducted a study using 37,869 patients with NHL, 8,323 with Hodgkin's lymphoma, and 13,842 with chronic lymphocytic leukemia who were diagnosed between 1965 and 2004. The study included 236,408 matched controls and 613,961 first-degree relatives. The results indicated that people with celiac disease developed NHL at rates that were 5.35 times higher than non-celiacs, but that they faced no increased risk for developing Hodgkin’s lymphoma or chronic lymphocytic leukemia. In some good news, the doctors found that the NHL risk level for people whose celiac disease was diagnosed between 1995 and 2004 dropped to just 3.86 times greater than for non-celiacs. This is a significant improvement over the 13.2 times greater risk of NHL faced by people diagnosed with celiac disease between 1975 and 1984. However, the study also showed that siblings of celiac disease patients developed NHL at rates that were more than double those of the general population (2.03). Clearly, as diagnosis and treatment of celiac disease has improved, the risk levels for NHL have decreased. The study underscores the need for greater vigilance on the part of both doctors and patients regarding NHL, and for greater understanding of the mechanisms that influence the development of NHL in both celiacs and non-celiacs. As diagnosis and treatment and monitoring of celiac disease improves, and as understanding of NHL increases, it is likely that NHL risk levels for celiac patients will drop even further. Until then, celiac patients are encouraged to stay informed, stay vigilant, and to consult with a physician to keep on top of any developments that may influence risk levels for NHL. Journal of Gastroenterology, January 2009; pp 91-98.
  10. 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
  11. 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
  12. Celiac.com 04/13/2010 - A team of clinicians recently described a case of immune modulation by non-Hodgkin lymphoma in a patient with two primary intestinal T-Cell lymphomas and long-standing celiac disease. F. Mühr-Wilkenshoff, M. Friedrich, H. D. Foss, M. Hummel, M. Zeitz, and S. Daum made up the research team. They are variously affiliated with the Medical Clinic I, Gastroenterology, Rheumatology and Infectious Diseases, and with the Department of Pathology, Charité of the Campus Benjamin Franklin of University Medicine Berlin, Germany. About 20–30% of all non-Hodgkin lymphomas (NHLs) are gastrointestinal in nature. Of these gastrointestinal lymphomas, about 20–30% occur in small intestine The clinical team recently reported the case of a 72-year-old patient who had been diagnosed with celiac disease when he was 52-years old. The man had not followed a gluten-free diet, yet showed no evidence of enteropathy or celiac-associated antibodies, but still developed a jejunal T-cell lymphoma. Doctors resected the lymphoma due to perforation and treated the patient with four courses of IMVP-16. The patient began and maintained a strict gluten-free diet. Two years later, the patient appeared with weight loss and a clonally divergent refractory sprue type II with loss of antigen (CD8; T-cell receptor-) expression in intraepithelial lymphocytes. At this time, he showed high titers of celiac-associated antibodies, although he was on a strict GFD. The research team notes that the missing enteropathy under a gluten-containing diet supports the idea of immune suppression in malignant diseases, especially non-Hodgkin lymphoma. They also note that the fact that, even while maintaining a strict gluten-free diet, the patient developed refractory sprue type II, an early form of another independent T-cell lymphoma, along with celiac-associated antibodies, suggests that clonal intraepithelial lymphocytes might be stimulating antibody production. Thus, they conclude that isolated detection of celiac-associated antibodies in patients with celiac disease does not prove that patients have deviated from their gluten-free diets. Source: Digestion 2010;81:231–234 DOI: 10.1159/000269810
  13. Celiac.com 03/16/2010 - Enteropathy associated T-cell lymphoma (EATL) is a rare type of peripheral T-cell lymphoma that is commonly associated with celiac disease. A group at The Newcastle Lymphoma Group in the United Kingdom, evaluated data from newly diagnosed patients in Northern England and Scotland between 1994 and 1998, in search of increased overall survival (OS) rates and progression free survival (PFS) rates for EATL patients. Celiac disease (celiac disease) is the most common food intolerance disorder affecting Western civilization today. While most celiacs show an improvement in their health after initiating a gluten free diet, 2-5% of patients do not improve, and are thus considered to have refractory celiac disease (RCD). RCD is further classified into two categories, Type 1 with intraepithelial lymphocytes of normal phenotype, or as type 2 with clonal expansion of intraepithelial lymphocytes with an aberrant phenotype. Type 2 patients are expected to have a five year overall survival rate (OS) of 50%-58%, and most Type 2 RCD patients die from EATL. EATL generally affects older patients in their 60's or 70's, with a history of celiac disease or RCD, and is most frequently presented in the form of malabsorption along with abdominal pain. However, EATL is not exclusive to patients with celiac disease or RCD and has also been found in patients without a history of either. Standard treatments until now have included surgical resection, with or without anthracycline-based chemotherapy, or high-dose chemotherapy with autologous stem cell transplant (ASCT). Results of these treatments have been dismal, with the patient typically dying from disease related complications. Using a population-based setting, 26 EATL patients that qualified for intensive treatment were given the new aggressive treatment of, ifosfamide, vincristine, etoposide / methotrexate (IVE/MTX) & ASCT, and their results were compared to that of the historical group. Statistically there was no difference between the groups; all groups had similar age, sex and features at initial evaluation. For all patients treated with the historical cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) therapy, the average PFS rate was approximately three months, and the average OS rate was about seven months. However, the IVE/MTX - ASCT group showed a significantly higher five year PFS and OS compared to patients treated with the historical CHOP therapy. Additionally, patients treated with IVE/MTX - ASCT showed improvement in their remission rates, and had profound reduction of death rates compared to the group treated with the historical CHOP chemotherapy. Of the patients that were solely treated with surgery, none survived. While EATL has a somber outcome for most patients treated with conventional CHOP treatments, data collected from these tests reveal that the regime IVE/MTX – ASCT shows exceptional promise as a new treatment. It is recommended that EATL patients enter themselves into national studies like this one, to expand research data and to help explore potentially effective EATL treatment options. Source: DOI 10.1182/blood-2009-07-231324.
  14. 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
  15. Eur J Gastroenterol Hepatol 2006;18:187-194. Celiac.com 04/10/2006 - According to findings by Dutch researchers, celiac disease increases the risk of non-Hodgkin lymphoma—but to a lower level than once believed. Past celiac disease studies have indicated that there is a 30 to 40-fold increased risk of enteropathy-associated T-cell lymphoma, however, Dr. M. Luisa Mearin and colleagues in The Netherlands investigated the frequency of celiac disease in two large European populations—one was a control group and the other was a group of non-Hodgkin lymphoma patients—and found that 1.2% of the non-Hodgkin lymphoma patients had celiac disease compared to 0.5% of the controls. After adjusting for age and sex differences between the two groups they found that celiac disease patients had a 2.6-fold increase risk of getting non-Hodgkin lymphoma, and this increased risk was only associated with patients who had been diagnosed prior to the study, and not in those with “silent” celiac disease which was found during the study. The odds of T-cell type small bowel lymphoma in celiac disease patients was estimated to be 28 times higher than for other localizations. The researchers conclude that celiac disease patients have a significantly increased risk of developing non-Hodgkin lymphoma, but the association is lower than previously thought. Celiac disease is mainly associated with T-cell small bowel lymphoma which is, in general, a rare condition.
  16. Gut 2005;54:54-59. Celiac.com 01/20/2005 - A link between untreated celiac disease and a rare enteropathy-type T-cell lymphoma (ETTL) has been well established by several studies. According to Dr. Karin Ekstrom Smedby of the Karolinska Institute in Stockholm and colleagues, there is also an increase in the prevalence of other types of lymphomas in those with celiac disease, such as B cell and non-intestinal lymphomas. In their study the researchers reviewed and reclassified 56 cases of malignant lymphomas that occurred in 11,650 hospitalized celiac disease patients in Sweden. The observed numbers of lymphoma subtypes were compared with those expected in the Swedish population. The researchers discovered that a majority of the lymphomas were not intestinal T-cell lymphomas, but were B-cell non-Hodgkin lymphoma (NHL). In addition, 44% of the patients with B cell NHL had a history of other autoimmune/inflammatory diseases. As expected, the relative risks for T-cell NHL and primary gastrointestinal lymphomas were markedly increased. According to the researchers: "Most lymphomas complicating coeliac disease are indeed related to the disease and are not of the ETTL-type. There was a remarkable aggregation of autoimmune/inflammatory disorders, female sex, coeliac disease, and B cell lymphoma."
  17. JAMA 2002;287:1413-1419. Celiac.com 04/12/2002 - According to a report published in the March 20th issue of the Journal of the American Medical Association, people with celiac disease are three times more likely to develop non-Hodgkin lymphoma (NHL) than the normal population. Dr. Carlo Catassi and colleagues from the University of Maryland in Baltimore compared the prevalence of celiac disease in 653 NHL patients with more than 5,000 healthy control subjects to determine the NHL-celiac disease occurrence rate. The results indicate that 1% of NHL patients also have celiac disease, in comparison with 0.42% of the healthy controls. Adjustments were made for age and sex, and the final results indicate that the odds ratios for a patient with celiac disease of developing NHL are: 3.1 for all types of NHL, 16.9 for gut NHL, and 19.2 for T-cell NHL. The overall risk, however, for someone with celiac disease developing NHL is only 0.63%. The researchers do not feel that their findings support mass screening for celiac disease, but they do feel that selected NHL patients should be screened for celiac disease. We would also like to add that these findings support the screening of people with celiac disease for NHL, which was not directly addressed by the report.
  18. Eur J Gastroenterol Hepatol 2000;12:645-648. Celiac.com 08/13/2000 - According to Drs. Simon D. Johnston and R.G. Peter Watson from Royal Victoria Hospital in Belfast, Northern Ireland, UK, the incidence of undiagnosed celiac disease is higher among those with small bowel lymphoma, as reported in the June issue of the European Journal of Gastroenterology and Hepatology. According to the researchers: It is not clear whether the increased risk of small bowel lymphoma seen in typical celiac disease also applies to unrecognized or screening-detected celiac patients. To find an answer, they retrospectively identified 69 cases of small-bowel adenocarcinoma and 69 cases of small-bowel lymphoma from five pathology laboratories in Northern Ireland. From a group composed of one patient with known celiac disease, and 12 with previously unrecognized celiac disease, the clinical presentation of adenocarcinoma and lymphoma patients was similar, but perforation was much more common among lymphoma patients. Further, 13 of the lymphoma patients, but none of the adenocarcinoma patients, had villous atrophy at a distant site, all of which were enteropathy-associated T-cell lymphomas. According to the researchers: Comparing the small-bowel lymphoma group to our random sample of the general Northern Ireland population as controls, the odds ratio of 15.72 for unrecognized celiac disease in the small-bowel lymphoma group, clearly indicates that there is an increased risk of unrecognized celiac disease among small-bowel lymphoma patients. Additionally, (s)ince a protective role for a strict gluten-free diet has been demonstrated, it follows that every effort should be made to diagnose celiac disease at every opportunity and raises the issue of whether population screening for celiac disease should be carried out.
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