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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

Celiac.com 05/27/2023 - Malignancy must be a concern for all of us with celiac disease. The association between increased mortality in celiac disease due to malignant disease has been known since 1962(1) . Subsequent studies have confirmed various types of malignancies occurring in celiac patients with the most frequent being lymphomas, which account for 51-72% of celiac disease-associated malignancy(2, 3) . Both small bowel lymphomas and adenocarcinoma, the most frequent celiac disease-associated malignancies, typically arise in the jejunum but also are found in the duodenum and ileum(2) . Other sites of carcinoma found in greater than expected numbers have been the mouth, pharnyx, lung, breast, and testes2, 3. Celiac disease-associated cancer is found in both child and adult celiacs(2). Enteropathy associated T-cell lymphoma (EATL) of the small bowel is the major lymphoma associated with celiac disease(2, 4). This type of non-Hodgkin lymphoma appears to be primarily associated with celiac disease and is known to increase in people with celiac disease who are 50-70 years of age(4) . There appears to be two forms of EATL, and one of them may originate during refractory celiac disease(2,4). Abnormalities in refractory celiac disease lymphocytes are similar to those of this form of EATL(3) . While the majority of patients with celiac disease have improved symptoms with a strict gluten-free diet, those with refractory celiac disease may be non-responsive because of complications due to the development of EATL(5). The Importance of a Gluten-Free Diet The exact rate of malignancy in celiac disease is unknown since much of the silent or asymptomatic form of the disease remains undiagnosed(5) . Also, the celiac disease status of patients with established lymphoma may never be determined, or may be missed at examination(2, 5). However, European studies have shown an increased mortality rate due to malignancies in celiac disease as high as two to nearly four times that of the non-celiac disease population(2) . Most deaths occurred in the first 3-4 years after diagnosis. Several studies have demonstrated the protective effect of a strict gluten-free diet against malignancy(2, 5) . There appears to be a clear correlation between increased cancer rates (comparing the celiac disease and non-celiac disease populations) and the amount of gluten ingestion. In one of the studies, the excess morbidity of patients on a strict gluten-free diet was only 1.2 compared to 10.7 in patients on a normal (gluten-containing) diet(5). After 5 years or more on a strict gluten-free diet, there appears to be no significant increase in the overall cancer risk compared to the non-celiac disease population. Furthermore, a strict gluten-free diet appears to specifically reduce EATL(6). The Importance of Early Diagnosis Conferring the protective effect of a gluten-free diet with early diagnosis is important in malignancies like lymphoma, which has a poor prognosis(5). The expected five-year survival of advanced small bowel lymphoma is 25-30%; that of intestinal T cell lymphoma is only about 25%7 . Furthermore, gastrointestinal lymphomas often are presented as high grade (i.e., more advanced) malignancy, and are often widespread(5,6). Unfortunately some cases of celiac disease are not diagnosed until presentation of lymphoma. T-cell lymphomas most often arouse the suspicion of undiagnosed celiac disease, but B-cell lymphomas exist in celiac disease as well(8). However, the diagnosis of lymphoma can be difficult to ascertain due to non-specific symptoms or symptoms similar to celiac disease(5,7). Presenting features of gastrointestinal lymphoma are similar to that of uncomplicated celiac disease; "Unexplained deterioration, abdominal pain, weight loss, severe muscle weakness, lymphadenopathy (disorder of the lymph nodes), abdominal mass and pyrexia (fever) should arouse suspicion of lymphoma."(5). Some patients may also have intestinal obstruction, perforation, or bleeding. Furthermore, patients with small bowel tumors (including adenocarcinoma) may present with abdominal pain, anemia, bleeding, weight loss, or obstruction(2, 7). Therefore, Ruskone-Fourmestraux and Rambaud suggest that "the diagnosis of coeliac disease must be made as early as possible and the diet commenced, even in asymptomatic subjects, after detailed and complete patient information."(6). Once diagnosed, therapy for lymphoma or adenocarcinoma may include surgery (such as resection of a portion of the intestine), chemotherapy, and/or radiotherapy(5). Regarding cancer, there is both good and bad news for those of us with celiac disease. While we have an increased risk of cancer, the risk is still very small for most celiacs. The symptoms of gastrointestinal cancers, and especially small bowel cancers, are similar to those of celiac disease itself. It appears, however, that with the exception of refractory celiac disease, a strict gluten-free diet over time may remove the increased chance of cancer due to celiac disease. Undeniably, our vigilant adherence and attitude toward the gluten-free lifestyle must be a mainstay and we must be up to the challenge. References: Gough KR, Read AE, Naish JM. 1962. Intestinal reticulosis as a complication of idiopathic steatorrhea. Gut 3: 232-39. Green PHR, and Jabri B. 2002. Celiac disease and other precursors to small-bowel malignancy. Gastroenterol Clin N Am 31:625-39. Seraphin P, and Mobarhan S. 2002. Mortality in patients with celiac disease. Nutrition Rev 60: 116-8. Catassi C, et al. 2002. Risk of non-Hodgkin lymphoma in celiac disease. JAMA 287:1413-9. Holmes GKT. 2002. Coeliac disease and malignancy. Digest Liver Dis 34:229-37. Ruskone-Fourmestraux A, and Rambaud JC. 2001. Gastrointestinal lymphoma: prevention and treatment of early lesions. Best Practice & Res Clin Gastroenterol 15:337-54. Gill SS, Heuman DM, and Mihas AA. 2001. Small intestinal neoplasm. J Clin Gastroenterol 33: 267-82. Freeman H, Lemoyne M, and Pare P. 2002. Coeliac disease. Best Pract & Res 16:37-49.

Celiac.com 09/20/2021 - People with celiac disease face an increased risk of death, in part due to cancer. Most studies investigating this cancer risk involved patients diagnosed before widespread increases in celiac disease diagnosis rates and access to gluten-free food. A team of researchers recently conducted a population-based study to assess the risk of cancer for people with celiac disease. For their study, the team used the Epidemiology Strengthened by histoPathology Reports in a Swedish cohort to gather data from all celiac disease patients in Sweden, with celiac disease defined as duodenal/jejunal villus atrophy. They then matched each patient by age, sex, and county to five or fewer control subjects. Then, following patients from diagnosis until first cancer, or by December 31, 2016, they calculated hazards ratios using the stratified Cox proportional hazards model. Of nearly 50,000 celiac patients, 64% were diagnosed with celiac disease since 2000. After a median follow-up of 11.5 years, the incidence of cancer was 6.5 and 5.7 per 1000 person-years in celiac disease patients and controls, respectively. The risk of cancer rose overall, but it was most sharply elevated in the first year after celiac disease diagnosis, and not later on, although the risks of hematologic, lymphoproliferative, hepatobiliary, and pancreatic cancers remained. Risk levels were highest for people diagnosed with celiac disease after age 60 years of age, while those diagnosed before age 40 faced no such increase. Lastly, the cancer risk was similar among those diagnosed with celiac disease before or after the year 2000. The team's data showed an overall rise in cancer risk for celiac disease patients, even in recent years. However, the risk increase is only for those diagnosed with celiac disease after age 40, and then mostly within the first year of diagnosis. This is one of the first studies to give a solid picture of overall cancer risks for people with celiac disease. Stay tuned for more on this and related stories. Read more in Clinical Gastroenterology and Hepatology The research team included Benjamin Lebwohl; Peter H.R. Green; Louise Emilsson; Karl Mårild; Jonas Söderling; Bjorn Roelstraete; and Jonas F. Ludvigsson. They are variously affiliated with the Celiac Disease Center, Department of Medicine, Columbia University Medical Center, New York, New York; the Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, New York; the Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, Gothenburg, Sweden; the Department of Pediatric Gastroenterology, Queen Silvia Children’s Hospital, Gothenburg, Sweden; the Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden; and the Clinical Epidemiology Division, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden. 12/06/2021 - A correction was made to the article "64% were diagnosed with celiac disease since 2000."

Celiac.com 11/21/2020 - A light snow is falling and the air carries a December chill. I am reminded of my brother's death on a similar day eleven short years ago. During the preceding year, doctors refused to test my brother for celiac disease following his diagnosis with lymphoma. Their stated reasons were that celiac disease is too rare to be considered, he showed none of the classic signs of celiac disease, and his lymphoma, a separate and more serious illness, needed to be addressed first. One physician even stated that the gluten-free diet was nutritionally inadequate and it would deprive my brother of the meal-time pleasures of a regular diet "during the time he has left." Celiac disease, I was told, could be investigated after the lymphoma was resolved. Concurrent and subsequent discussion revealed that my brother's death was the resolution they expected. At that time, only a small pocket of researchers, along with a group of informed physicians (and others) who managed to stay current with the medical literature regarding celiac disease, were aware that it can be a large underlying contributor to lymphoma and other cancers, and institution of a gluten-free diet can provide some important benefits to those battling cancers in the context of celiac disease. Aside from the obvious objections to claiming that Jack would enjoy food—any food—during multiple courses of chemo and radiation therapy is suspect, and that it might have been valuable to forestall prophesying his death until after these treatments were at least begun, a number of larger objections arise out of these physicians' confident disregard for the medical and scientific literature. The pervasive attitude was one that trivialized celiac disease and dismissed any other form of gluten sensitivity. Fortunately, an emerging paradigm is supplanting these outdated prejudices and the last decade has witnessed huge gains in physicians' and public awareness of celiac disease. Students of celiac disease, dermatitis herpetiformis, and gluten syndrome (non-celiac gluten sensitivity) know that gluten induced illness is far from rare and can manifest in an enormously wide range of signs and symptoms. Turn-of-the-century publication of multi-center research has established that celiac disease, far from being rare, is found in about one percent of the U.S. population. Most of these individuals also lacked the classic signs and symptoms of celiac disease. Further, the rate of gluten syndrome is at least ten times that of celiac disease, and gluten antibodies are very frequently found among those with neurological disease. The research team headed by Marios Hadjivassiliou at the Royal Hallamshire Hospital in Sheffield, England has shown that more than half of patients suffering from neurological ailments of unknown origin are gluten sensitive. (Most neurological diseases are of unknown origin.) Another group working at the University of Alberta, led by B. Yacyshyn, reported leaky gut in twenty five percent of a group of twenty multiple sclerosis patients, so gluten is an important suspect in at least some cases of one neurological disease that is fairly well characterized. Another report of just two patients shows that a gluten-free diet can abolish islet cell antibodies that destroy insulin producing cells in the pancreas. These antibodies are the basis of type I diabetes. Other reports show that obesity can be induced by gluten and mediated by a gluten-free diet. Still others report amelioration of psychiatric illnesses, behavioral abnormalities, and/or learning disabilities as the result of a gluten-free diet. Most students of the gluten syndrome are learning about this wide range of signs, symptoms, and associated illnesses. Many of us have experienced improved general health along with subtle improvements across a range of modalities and perceptions. Many of us suspect that these changes exceeded what could be expected to arise from improved general health. When learning about the protean manifestations of gluten induced illness, those suspicions are affirmed. It is through sharing this learning that celiac disease and gluten syndrome have begun a shift from the fringes of our culture to widespread, mainstream awareness of gluten induced illness in all its manifestations. Much of the research community involved in celiac disease has led the way. Increasingly informed (largely through the Internet) patients and their support groups have moved awareness of celiac disease from obscurity to mainstream in little over a decade. Recognition of gluten syndrome lags far behind celiac disease, but is increasing rapidly. The final, large step in this process of enlightenment is to assist those struggling, straggling, overworked general practitioners get up to speed on this most common illness. These are the individuals who continue to ignore the literature on this topic. They confidently dismiss the possibility of celiac disease or gluten syndrome on the basis of an out-dated, often prejudiced, largely erroneous understanding of gluten-induced illness as it was taught during their training. The sadness of my brother's passing has abated to the point where I can now think and talk about it without the sense of emptiness and loss that comes of knowing that I won't see him again—and without the anger and frustration borne of the widespread medical ignorance at the general practitioner level. Today, I can freely share my brother's painful, final journey in the hope that it will hasten the abolition of the medical ignorance and arrogance that helped speed his untimely death. I still struggle with the memory that my brother's doctors complained to Jack about my badgering them to test him for celiac disease. They complained to a dying man about my annoying letters, while they ignored my repeated pleas that he be tested for celiac disease. I am still chagrined by their dismissal of research showing a substantial genetic component in celiac disease, knowing full well that I, his brother, had previously been diagnosed with celiac disease. During the eleven years since my brother's death, an emerging awareness of gluten mediated illness has blossomed throughout the industrialized world and has reached tentacles into the third world. Although knowledge of the health hazards of gluten is spreading world-wide, recognition of gluten's threat to human health is still an emerging paradigm. Another decade of dedicated effort on the part of support groups, researchers, and others, should have celiac disease appearing frequently in the differential diagnosis for an enormously wide range of signs and symptoms, with gluten syndrome following on its heels. And perhaps a gluten-free, dairy-free diet will soon be standard fare for most cancer patients, freeing their immune systems to battle malignancies rather than gluten.

Celiac.com 05/24/2019 (originally published 10/08/2010) - I need to do some studying about the individual recommendations, as I am still not well-versed in many of these alternative therapies. However, I think I can dig most of it out now with what I have picked up from others over the years. It all makes sense to me through my newly-opened eyes. We don’t talk much about the virus(es) involved in canine lymphoma, even though it is known to be viral in most other affected species. The big question is whether pleomorphic bacteria are also involved, as they are now suspected to be by many researchers in human cases of Hodgkin’s lymphoma. This makes sense as viruses and bacteria act as a “tag-team” in both normal adaptation in the day-to-day life of the cell as well as the “over-adaptive” processes we call disease, including “autoimmune disease” and cancer. Bacteria are interacting with the mitochondria, driving the cellular metaplasia (and “precancerous” changes) while viruses are interacting with the nucleus, driving the rapid cellular division that characterizes cancer. The question then becomes: What is the real reason “malignancy” occurs? What is/are the factors in this syndrome that make one tumor become more aggressive than another? Is it an additional virus or pleomorphic bacteria that completes picture…a helper virus as some describe it? To a degree, this is academic because the approach is the same- we need to stop doing what we are doing that has caused this in the first place. Cancer is simply a spectrum disorder and the natural consequence of our not dealing properly with “phase two”—the “autoimmune diseases”, in which the immune system is desperately trying to get this mess under control so that the cells and their adaptive residents (viruses and bacteria) don’t feel compelled to start forming tumors (cocoons) to protect themselves from the toxic environment in which they find themselves. “Disease”, including cancer, is no longer a mystery at all, is it? The only differences between one disease and another is the different viruses and bacteria involved (many of which are lurking in the DNA, passed down through the generations), the various triggers (toxic insults we throw at them), and the cell/organ involved. The age and rapidity of onset is determined by concurrent illnesses (malnutrition/leaky gut, hypothyroidism, adrenal and pituitary dysfunction, gonadal insufficiencies, etc.), most of which can be connected to the same leaky gut, malnutrition, and toxic insults that we believe contributed to the cancer. So…the logical approach is to reestablish wellness as quickly as possible by healing the gut (the removal of gluten, et al), providing a biologically appropriate, eliminating as many environmental toxins as possible (e.g. fluoride, pesticides), identifying and treating the concurrent illnesses (e.g. hypothyroidism), and then addressing the viruses and bacteria that are involved in the process. (Knowing what we now know about pleomorphic bacteria and cancer, could antibiotics help?…or would they do more harm than good by forcing these bacteria into adapting further???) Thankfully, by taking the steps outlined above, we often won’t have to address the viruses and bacteria specifically as they will be satisfied with our efforts and go back to doing what they were doing before we bombarded them with all of the toxic insults, which was helping the cells to adapt to change. But in the case of cancer, we have our work cut out for us. The individual with cancer has had some degree of immune failure, at least at a local level, for cancer to have occurred. In these “end game cases”, we have to do everything right, which brings into question the logic behind the use of carcinogenic chemotherapy. In the cases of overwhelming bacterial infections (which primarily occur because of weakened immunity and toxic insults), we frequently have to pull the afflicted individual from the fire by using powerful antibiotics, killing the “offending” bacteria, which were often normal residents of the body before they were forced into replicating at a pathological rate, due to the toxic environment in which they found themselves, and often in a body that is repeatedly insulted by malnutrition, pollutants (e.g. air pollution, cigarette smoke, dietary insults), and poor lifestyle choices. Whose fault is it that these normally-helpful microorganisms rebelled??? So…once again…in the case of cancer, do we occasionally need to pull out the big guns that bring down the strongholds of these scapegoats we call viruses and bacteria? Do we sometimes need to blast their cocoons with harsh and potentially carcinogenic chemotherapy in order to pull the afflicted from the fire in the same way that antibiotics, anti-fungals and anti-virals are used to rescue the less-afflicted??? I contend that we wouldn’t if…IF…we could correct enough of the underlying causes. But…that’s a big “if”. Can we eliminate enough carcinogens with our patients living in polluted cities? Can we provide them with a perfect diet to reverse the catastrophic malnutrition from which many suffer? Can we give them all purified water, free of the fluoride and other toxins commonly found in the general water supply? Can we convince them to leave a highly polluted city (e.g. NYC, Boston, LA, Atlanta, Phoenix, Mobile) that is assaulting them with more than enough carcinogens and neurotoxins to place them firmly in the predicament in which they find themselves? Can we give them enough antioxidants, nutriceuticals, essential oils, and homeopathic remedies to undo the years of physical abuse and poor lifestyle choices that led to this calamity? Sadly, in most cases, the answer is “no”. But that doesn’t mean we can’t try. That is the main difference between us and the classically-trained, allopathic doctor: We are at least considering our role in this and making use of what we learned in basic Sciences about how the body truly works. Isn’t it amazing how the first years of medical school (the basic Sciences of Anatomy, Histology, Pathology, Physiology, etc.) are almost completely wiped out by the final years of clinical work? Suddenly, we have a pill for everything and are focusing on drug dosages and interactions, having completely forgotten WHY the body does what it does. Is this brain-washing an accident or intentional on some level? One has to wonder. The true role of our residential organisms (viruses, bacteria, fungi and parasites) is one of the most amazing things I have learned in the past ten years. Once we grasp (or re-grasp) the adaptive process—the fact that there are living entities that move in and out of cells reporting on and adapting to the outside environment, the mystique of medicine disappears and “greater things” become possible. But the limitations in the lives of the afflicted individual are both real and saddening. For them, we have to do exactly what we are doing here: Come up with the best compromise. In the case of cancer, is the tumor/process life-threatening enough to warrant further intoxicating the body? Will a solitary tumor or enlarged lymph nodes kill the patient? Is the systemic cancer like lymphoma causing enough organ dysfunction to justify the use of immunosuppressive drugs (e.g. prednisone) and carcinogenic chemicals, both of which are illogical once we know that the true underlying “causes” are viruses and bacteria that have simply (and visibly) rebelled in response to our own wrong-doing, including trashing our immune system so thoroughly that it can’t control the process even without the addition of more immune-suppressing drugs? We are our own worst enemy. But…at least we know that now.

Celiac.com 05/22/2019 - What are the risks for lymphoma and gastrointestinal cancer in patients 55 years and older with newly diagnosed adult-onset celiac disease? Researchers and clinicians have reported connections between celiac disease and the development of certain lymphoid and gastrointestinal (GI) cancers, but there just isn't much good data. Without good data, it's impossible to develop effective evidence-based follow-up protocols. In an effort to develop better information on the subject, a team of researchers recently set out to determine relative (RR) and absolute risks of lymphoma and GI carcinoma for newly diagnosed adult celiac patients. The research team included Tom van Gils, Petula Nijeboer, Lucy IH Overbeek, Michael Hauptmann, Daan AR Castelijn, Gerd Bouma, Chris JJ Mulder, Flora E van Leeuwen, and Daphne de Jong. They are variously affiliated with the Celiac Center Amsterdam, Department of Gastroenterology and Hepatology, VU University Medical Center, Amsterdam, the Netherlands; the Foundation PALGA (The Nationwide Network and Registry of Histo- and Cytopathology in the Netherlands), Houten, the Netherlands; the Department of Epidemiology and Biostatistics, the Netherlands Cancer Institute/Antoni van Leeuwenhoek, Amsterdam, the Netherlands; and the Department of Pathology, VU University Medical Center, Amsterdam, the Netherlands. To assess RR with cases (lymphoma or GI carcinoma) and controls--melanoma or basal cell carcinoma diagnosed from 1994–2014, the team conducted a case-control design using the Dutch nationwide population-based pathology database (PALGA). Among this group, the team identified patients with prior histologically proven or simultaneously diagnosed with the malignancy. The team found celiac disease in a total of 349 of 301,425 cases (0.1%) and 282 of 576,971 (0.05%) control subjects. Adults diagnosed with celiac disease had a substantially higher risk of T-cell lymphoma, mainly enteropathy-associated T-cell lymphoma (EATL). Clinicians should look out for EATL (both intestinal and extra-intestinal) and small bowel adenocarcinoma in patients with celiac disease diagnosed at 50 years of age or later. Although most often synchronously diagnosed, risk of T-cell lymphoma 1 year or more after celiac disease diagnosis was still elevated at 12.7 (95% CI 7.6–21.3). Other celiac disease-associated malignancies were small bowel adenocarcinoma, with RR of 11.9 (95% CI 8.2–17.2), and esophageal squamous cell carcinoma (RR = 3.5 (95% CI 2.1–5.8)). Absolute risks of developing these cancers were relatively low. Celiac disease did not show any higher risk of developing other types of lymphomas and GI carcinomas. Read more in the United European Gastroenterology Journal

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.

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