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Chris J. J. Mulder - One of the best experts on this subject based on the ideXlab platform.
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serendipity in refractory celiac disease full recovery of duodenal villi and clinical symptoms after fecal microbiota transfer
Journal of Gastrointestinal and Liver Diseases, 2016Co-Authors: Yvette H Van Beurden, Chris J. J. Mulder, Tom Van Gils, Nienke A Van Gils, Zain Kassam, Nieves ApariciopagesAbstract:Treatment of refractory celiac disease type II (RCD II) and preventing the development of an enteropathy associated T-Cell Lymphoma in these patients is still difficult. In this case report, we describe a patient with RCD II who received fecal microbiota transfer as treatment for a recurrent Clostridium difficile infection, and remarkably showed a full recovery of duodenal villi and disappearance of celiac symptoms. This case suggests that altering the gut microbiota may hold promise in improving the clinical and histological consequences of celiac disease and/or RCD II.
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evaluation of cladribine treatment in refractory celiac disease type ii
World Journal of Gastroenterology, 2011Co-Authors: Greetje J Tack, Otto Visser, Wieke H M Verbeek, Abdulbaqi Altoma, Dirk J Kuik, Marco W J Schreurs, Chris J. J. MulderAbstract:AIM: To evaluate cladribine [2-chlorodeoxyadenosine (2-CdA)] therapy in refractory celiac disease (RCD) II. METHODS: An open-label cohort-study of RCD II patients treated with 2-CdA was performed between 2000 and 2010. Survival rate, enteropathy associated T-Cell Lymphoma (EATL) occurrence, clinical course, and histological and immunological response rates were evaluated. RESULTS: Overall, 32 patients were included with a median follow-up of 31 mo. Eighteen patients responded well to 2-CdA. Patients responsive to 2-CdA had a statistically significant increased survival compared to those who were unresponsive. The overall 3- and 5-year survival was 83% in the responder and 63% and 22% in the non-responder group, respectively. The overall 2-year clinical, histological and immunological response rates were 81%, 47% and 41%, respectively. Progression into EATL was reported in 16%, all of these patients died. CONCLUSION: Treatment of RCD II with 2-CdA holds promise, showing excellent clinical and histological response rates, and probably less frequent transition into EATL.
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the spectrum of celiac disease epidemiology clinical aspects and treatment
Nature Reviews Gastroenterology & Hepatology, 2010Co-Authors: Greetje J Tack, Wieke H M Verbeek, Marco W J Schreurs, Chris J. J. MulderAbstract:Celiac disease is a gluten-sensitive enteropathy that affects people of all ages worldwide. This disease has emerged as a major health-care problem, as advances in diagnostic and screening methods have revealed its global prevalence. Environmental factors such as gluten introduction at childhood, infectious agents and socioeconomic features, as well as the presence of HLA-DQ2 and/or HLA-DQ8 haplotypes or genetic variations in several non-HLA genes contribute to the development of celiac disease. Growing insight into the variable clinical and histopathological presentation features of this disease has opened new perspectives for future research. A strict life-long gluten-free diet is the only safe and efficient available treatment, yet it results in a social burden. Alternative treatment modalities focus on modification of dietary components, enzymatic degradation of gluten, inhibition of intestinal permeability and modulation of the immune response. A small group of patients with celiac disease (2-5%), however, fail to improve clinically and histologically upon elimination of dietary gluten. This complication is referred to as refractory celiac disease, and imposes a serious risk of developing a virtually lethal Enteropathy-Associated T-Cell Lymphoma.
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enteropathy associated t cell Lymphoma and its precursor lesions
Best Practice & Research in Clinical Gastroenterology, 2010Co-Authors: Jolanda M Van De Water, Saskia A G M Cillessen, Otto Visser, Wieke H M Verbeek, Chris J L M Meijer, Chris J. J. MulderAbstract:Enteropathy Associated T-Cell Lymphoma (EATL) is an intestinal tumour of intra-epithelial lymphocytes. Based on morphology, immunohistochemistry and genetic profile EATL can be divided into two groups. EATL type I is a large cell Lymphoma which is highly associated with Coeliac Disease (CD) and mostly presents with malabsorption, weight loss and CD-related symptoms. EATL type II consists of small to medium-sized cells and presents often with obstruction or perforation of the small bowel. This type of EATL has no known association with CD. When EATL has been diagnosed a thorough diagnostic work-up is needed. This work-up preferably includes video capsule enteroscopy (VCE), double-balloon enteroscopy (DBE), computed tomography (CT) combined with 18F-fluorodeoxyglucose positron emission tomography scan (18F-FDG-PET scan) if possible and magnetic resonance enteroclysis (MRE). Nowadays, most EATL patients are treated with chemotherapy mostly preceded by resection of the tumour and followed by stem cell transplantation. Despite these therapies outcome of EATL remains very poor with a 5-year survival of 8–20%. In order to improve survival prospective multicentre trials, studying new therapies are needed. The combination of chemotherapy, monoclonal antibodies and/or apoptosis inducing small molecules might be a potential treatment for EATL in the (nearby) future.
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The Value of Double-Balloon Enteroscopy in Patients With Refractory Celiac Disease
The American Journal of Gastroenterology, 2007Co-Authors: M Hadithi, Chris J. J. Mulder, Abdulbaqi Al-toma, Joost J. Oudejans, Adriaan A. Van Bodegraven, Maarten A. J. M. JacobsAbstract:OBJECTIVE: Patients with refractory celiac disease can develop Enteropathy-Associated T-Cell Lymphoma (EATL) or ulcerative jejunitis. Double-balloon enteroscopy allows examination of the small bowel. We prospectively assessed the value of this technique in patients with refractory celiac disease in a tertiary referral center. METHODS: Small bowel enteroscopy was performed in a total of 21 consecutive patients for lesions like ulcerations (high risk). Biopsy specimens were taken from such lesions and from examined small bowel at three different levels of scope insertion depth. Tissue specimens were evaluated for the modified Marsh classification and for the presence of EATL. RESULTS: Twenty-four procedures were successfully performed without complications. EATL was found in five patients (24%, 95% CI 10-45%) as circumferential, discrete, or confluent ulcerations. In three of them, Marsh III was found while in the other two patients with EATL Marsh I was found. Another two patients (9%, 95% CI 2-28%) had ulcerative jejunitis in the absence of EATL and histology was compatible with Marsh III. In the remaining 14 patients (54%, 95% CI 35-73%), no high-risk lesions were found. Double-balloon enteroscopy could exclude the presence of EATL in four patients that was suggested by abdominal computerized tomography. CONCLUSIONS: Complications of refractory celiac disease like ulcerative jejunitis or EATL could efficiently be detected or excluded by double-balloon enteroscopy. This technique should be reserved for patients with refractory celiac disease or patients with a past history of EATL.
Wieke H M Verbeek - One of the best experts on this subject based on the ideXlab platform.
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auto sct in refractory celiac disease type ii patients unresponsive to cladribine therapy
Bone Marrow Transplantation, 2011Co-Authors: Greetje J Tack, Wieke H M Verbeek, Abdulbaqi Altoma, Marielle J Wondergem, A Schmittel, M V Machado, F Perri, Gert J Ossenkoppele, Peter C Huijgens, Marco W J SchreursAbstract:Autologous hematopoietic SCT (auto-SCT) has been effective therapy for refractory disease, in both malignancies and severe autoimmune diseases. It seems feasible and safe for refractory celiac disease (RCD) type II, although long-term results have not been evaluated yet. With current therapies, progression into Enteropathy-Associated T-Cell Lymphoma (EATL) occurs in 60-80% patients, with a high mortality rate. Therefore, it is important to evaluate new treatment strategies. Between March 2004 and February 2010, 18 RCD II patients were evaluated for auto-SCT preceded by conditioning with fludarabine and melphalan, as a consequence of unresponsiveness to cladribine therapy. Adverse events, survival rate, EATL development and change in clinical, histological and immunological course were monitored. Thirteen patients were transplanted successfully and followed up for >2 years, 4-year survival rate was 66%. Only one patient died because of transplant-related complications. The majority of patients showed an impressive clinical improvement and five a complete histological remission. In five patients, auto-SCT could not be performed; they all died with a median survival of 5.5 months. EATL was observed in one transplanted patient, only after 4 years of follow-up. Auto-SCT after conditioning with high-dose chemotherapy in RCD II patients unresponsive to cladribine therapy is feasible and seems promising.
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evaluation of cladribine treatment in refractory celiac disease type ii
World Journal of Gastroenterology, 2011Co-Authors: Greetje J Tack, Otto Visser, Wieke H M Verbeek, Abdulbaqi Altoma, Dirk J Kuik, Marco W J Schreurs, Chris J. J. MulderAbstract:AIM: To evaluate cladribine [2-chlorodeoxyadenosine (2-CdA)] therapy in refractory celiac disease (RCD) II. METHODS: An open-label cohort-study of RCD II patients treated with 2-CdA was performed between 2000 and 2010. Survival rate, enteropathy associated T-Cell Lymphoma (EATL) occurrence, clinical course, and histological and immunological response rates were evaluated. RESULTS: Overall, 32 patients were included with a median follow-up of 31 mo. Eighteen patients responded well to 2-CdA. Patients responsive to 2-CdA had a statistically significant increased survival compared to those who were unresponsive. The overall 3- and 5-year survival was 83% in the responder and 63% and 22% in the non-responder group, respectively. The overall 2-year clinical, histological and immunological response rates were 81%, 47% and 41%, respectively. Progression into EATL was reported in 16%, all of these patients died. CONCLUSION: Treatment of RCD II with 2-CdA holds promise, showing excellent clinical and histological response rates, and probably less frequent transition into EATL.
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the spectrum of celiac disease epidemiology clinical aspects and treatment
Nature Reviews Gastroenterology & Hepatology, 2010Co-Authors: Greetje J Tack, Wieke H M Verbeek, Marco W J Schreurs, Chris J. J. MulderAbstract:Celiac disease is a gluten-sensitive enteropathy that affects people of all ages worldwide. This disease has emerged as a major health-care problem, as advances in diagnostic and screening methods have revealed its global prevalence. Environmental factors such as gluten introduction at childhood, infectious agents and socioeconomic features, as well as the presence of HLA-DQ2 and/or HLA-DQ8 haplotypes or genetic variations in several non-HLA genes contribute to the development of celiac disease. Growing insight into the variable clinical and histopathological presentation features of this disease has opened new perspectives for future research. A strict life-long gluten-free diet is the only safe and efficient available treatment, yet it results in a social burden. Alternative treatment modalities focus on modification of dietary components, enzymatic degradation of gluten, inhibition of intestinal permeability and modulation of the immune response. A small group of patients with celiac disease (2-5%), however, fail to improve clinically and histologically upon elimination of dietary gluten. This complication is referred to as refractory celiac disease, and imposes a serious risk of developing a virtually lethal Enteropathy-Associated T-Cell Lymphoma.
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enteropathy associated t cell Lymphoma and its precursor lesions
Best Practice & Research in Clinical Gastroenterology, 2010Co-Authors: Jolanda M Van De Water, Saskia A G M Cillessen, Otto Visser, Wieke H M Verbeek, Chris J L M Meijer, Chris J. J. MulderAbstract:Enteropathy Associated T-Cell Lymphoma (EATL) is an intestinal tumour of intra-epithelial lymphocytes. Based on morphology, immunohistochemistry and genetic profile EATL can be divided into two groups. EATL type I is a large cell Lymphoma which is highly associated with Coeliac Disease (CD) and mostly presents with malabsorption, weight loss and CD-related symptoms. EATL type II consists of small to medium-sized cells and presents often with obstruction or perforation of the small bowel. This type of EATL has no known association with CD. When EATL has been diagnosed a thorough diagnostic work-up is needed. This work-up preferably includes video capsule enteroscopy (VCE), double-balloon enteroscopy (DBE), computed tomography (CT) combined with 18F-fluorodeoxyglucose positron emission tomography scan (18F-FDG-PET scan) if possible and magnetic resonance enteroclysis (MRE). Nowadays, most EATL patients are treated with chemotherapy mostly preceded by resection of the tumour and followed by stem cell transplantation. Despite these therapies outcome of EATL remains very poor with a 5-year survival of 8–20%. In order to improve survival prospective multicentre trials, studying new therapies are needed. The combination of chemotherapy, monoclonal antibodies and/or apoptosis inducing small molecules might be a potential treatment for EATL in the (nearby) future.
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survival in refractory coeliac disease and enteropathy associated t cell Lymphoma retrospective evaluation of single centre experience
Gut, 2007Co-Authors: Abdulbaqi Altoma, Wieke H M Verbeek, M Hadithi, B M E Von Blomberg, C J J MulderAbstract:Background: Coeliac disease may be regarded as refractory disease (RCD) when symptoms persist or recur despite strict adherence to a gluten-free diet. RCD may be subdivided into types I and II with a phenotypically normal and aberrant intraepithelial T-Cell population, respectively. RCD I seems to respond well to azathioprine/prednisone therapy. RCD II is usually resistant to any known therapy and transition into Enteropathy-Associated T-Cell Lymphoma (EATL) is common. Aim: To provide further insight into RCD and the development of EATL, by reporting on long-term survival and risk of transition of RCD into EATL in a large cohort of patients with complicated coeliac disease. Design and Methods: Retrospective comparison of responses to therapy in four groups of patients with complicated coeliac disease: 43, RCD I; 50, RCD II (total), of whom 26 with RCD II developed EATL after a period of refractoriness to a gluten-free diet (secondary EATL) and 13 were EATL patients without preceding history of complicated coeliac disease (de novo EATL). Results: No coeliac-disease-related mortality was recognised in the RCD I group. The overall 5-year survival in the RCD I group it was 96%; in the RCD II (total) group was 58%; and in the RCD II group after developing EATL it was only 8%. The 2-year survival in the de novo EATL group was 20% versus 15% in secondary EATL group (p = 0.63). Twenty-eight (56%) of the 50 patients with RCD II died, 23 (46%) due to EATL, 4 due to a progressive refractory state with emaciation and 1 from neurocoeliac disease. Conclusion: Remarkably, no patient with RCD I developed RCD II or EATL within the mean follow-up period of 5 years (range 2–15 years). A total of 52% of the RCD II patients developed EATL within 4–6 years after the diagnosis of RCD II. More aggressive and targeted therapies seem necessary in RCD II and EATL.
Greetje J Tack - One of the best experts on this subject based on the ideXlab platform.
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auto sct in refractory celiac disease type ii patients unresponsive to cladribine therapy
Bone Marrow Transplantation, 2011Co-Authors: Greetje J Tack, Wieke H M Verbeek, Abdulbaqi Altoma, Marielle J Wondergem, A Schmittel, M V Machado, F Perri, Gert J Ossenkoppele, Peter C Huijgens, Marco W J SchreursAbstract:Autologous hematopoietic SCT (auto-SCT) has been effective therapy for refractory disease, in both malignancies and severe autoimmune diseases. It seems feasible and safe for refractory celiac disease (RCD) type II, although long-term results have not been evaluated yet. With current therapies, progression into Enteropathy-Associated T-Cell Lymphoma (EATL) occurs in 60-80% patients, with a high mortality rate. Therefore, it is important to evaluate new treatment strategies. Between March 2004 and February 2010, 18 RCD II patients were evaluated for auto-SCT preceded by conditioning with fludarabine and melphalan, as a consequence of unresponsiveness to cladribine therapy. Adverse events, survival rate, EATL development and change in clinical, histological and immunological course were monitored. Thirteen patients were transplanted successfully and followed up for >2 years, 4-year survival rate was 66%. Only one patient died because of transplant-related complications. The majority of patients showed an impressive clinical improvement and five a complete histological remission. In five patients, auto-SCT could not be performed; they all died with a median survival of 5.5 months. EATL was observed in one transplanted patient, only after 4 years of follow-up. Auto-SCT after conditioning with high-dose chemotherapy in RCD II patients unresponsive to cladribine therapy is feasible and seems promising.
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evaluation of cladribine treatment in refractory celiac disease type ii
World Journal of Gastroenterology, 2011Co-Authors: Greetje J Tack, Otto Visser, Wieke H M Verbeek, Abdulbaqi Altoma, Dirk J Kuik, Marco W J Schreurs, Chris J. J. MulderAbstract:AIM: To evaluate cladribine [2-chlorodeoxyadenosine (2-CdA)] therapy in refractory celiac disease (RCD) II. METHODS: An open-label cohort-study of RCD II patients treated with 2-CdA was performed between 2000 and 2010. Survival rate, enteropathy associated T-Cell Lymphoma (EATL) occurrence, clinical course, and histological and immunological response rates were evaluated. RESULTS: Overall, 32 patients were included with a median follow-up of 31 mo. Eighteen patients responded well to 2-CdA. Patients responsive to 2-CdA had a statistically significant increased survival compared to those who were unresponsive. The overall 3- and 5-year survival was 83% in the responder and 63% and 22% in the non-responder group, respectively. The overall 2-year clinical, histological and immunological response rates were 81%, 47% and 41%, respectively. Progression into EATL was reported in 16%, all of these patients died. CONCLUSION: Treatment of RCD II with 2-CdA holds promise, showing excellent clinical and histological response rates, and probably less frequent transition into EATL.
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the spectrum of celiac disease epidemiology clinical aspects and treatment
Nature Reviews Gastroenterology & Hepatology, 2010Co-Authors: Greetje J Tack, Wieke H M Verbeek, Marco W J Schreurs, Chris J. J. MulderAbstract:Celiac disease is a gluten-sensitive enteropathy that affects people of all ages worldwide. This disease has emerged as a major health-care problem, as advances in diagnostic and screening methods have revealed its global prevalence. Environmental factors such as gluten introduction at childhood, infectious agents and socioeconomic features, as well as the presence of HLA-DQ2 and/or HLA-DQ8 haplotypes or genetic variations in several non-HLA genes contribute to the development of celiac disease. Growing insight into the variable clinical and histopathological presentation features of this disease has opened new perspectives for future research. A strict life-long gluten-free diet is the only safe and efficient available treatment, yet it results in a social burden. Alternative treatment modalities focus on modification of dietary components, enzymatic degradation of gluten, inhibition of intestinal permeability and modulation of the immune response. A small group of patients with celiac disease (2-5%), however, fail to improve clinically and histologically upon elimination of dietary gluten. This complication is referred to as refractory celiac disease, and imposes a serious risk of developing a virtually lethal Enteropathy-Associated T-Cell Lymphoma.
Marco W J Schreurs - One of the best experts on this subject based on the ideXlab platform.
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auto sct in refractory celiac disease type ii patients unresponsive to cladribine therapy
Bone Marrow Transplantation, 2011Co-Authors: Greetje J Tack, Wieke H M Verbeek, Abdulbaqi Altoma, Marielle J Wondergem, A Schmittel, M V Machado, F Perri, Gert J Ossenkoppele, Peter C Huijgens, Marco W J SchreursAbstract:Autologous hematopoietic SCT (auto-SCT) has been effective therapy for refractory disease, in both malignancies and severe autoimmune diseases. It seems feasible and safe for refractory celiac disease (RCD) type II, although long-term results have not been evaluated yet. With current therapies, progression into Enteropathy-Associated T-Cell Lymphoma (EATL) occurs in 60-80% patients, with a high mortality rate. Therefore, it is important to evaluate new treatment strategies. Between March 2004 and February 2010, 18 RCD II patients were evaluated for auto-SCT preceded by conditioning with fludarabine and melphalan, as a consequence of unresponsiveness to cladribine therapy. Adverse events, survival rate, EATL development and change in clinical, histological and immunological course were monitored. Thirteen patients were transplanted successfully and followed up for >2 years, 4-year survival rate was 66%. Only one patient died because of transplant-related complications. The majority of patients showed an impressive clinical improvement and five a complete histological remission. In five patients, auto-SCT could not be performed; they all died with a median survival of 5.5 months. EATL was observed in one transplanted patient, only after 4 years of follow-up. Auto-SCT after conditioning with high-dose chemotherapy in RCD II patients unresponsive to cladribine therapy is feasible and seems promising.
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evaluation of cladribine treatment in refractory celiac disease type ii
World Journal of Gastroenterology, 2011Co-Authors: Greetje J Tack, Otto Visser, Wieke H M Verbeek, Abdulbaqi Altoma, Dirk J Kuik, Marco W J Schreurs, Chris J. J. MulderAbstract:AIM: To evaluate cladribine [2-chlorodeoxyadenosine (2-CdA)] therapy in refractory celiac disease (RCD) II. METHODS: An open-label cohort-study of RCD II patients treated with 2-CdA was performed between 2000 and 2010. Survival rate, enteropathy associated T-Cell Lymphoma (EATL) occurrence, clinical course, and histological and immunological response rates were evaluated. RESULTS: Overall, 32 patients were included with a median follow-up of 31 mo. Eighteen patients responded well to 2-CdA. Patients responsive to 2-CdA had a statistically significant increased survival compared to those who were unresponsive. The overall 3- and 5-year survival was 83% in the responder and 63% and 22% in the non-responder group, respectively. The overall 2-year clinical, histological and immunological response rates were 81%, 47% and 41%, respectively. Progression into EATL was reported in 16%, all of these patients died. CONCLUSION: Treatment of RCD II with 2-CdA holds promise, showing excellent clinical and histological response rates, and probably less frequent transition into EATL.
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the spectrum of celiac disease epidemiology clinical aspects and treatment
Nature Reviews Gastroenterology & Hepatology, 2010Co-Authors: Greetje J Tack, Wieke H M Verbeek, Marco W J Schreurs, Chris J. J. MulderAbstract:Celiac disease is a gluten-sensitive enteropathy that affects people of all ages worldwide. This disease has emerged as a major health-care problem, as advances in diagnostic and screening methods have revealed its global prevalence. Environmental factors such as gluten introduction at childhood, infectious agents and socioeconomic features, as well as the presence of HLA-DQ2 and/or HLA-DQ8 haplotypes or genetic variations in several non-HLA genes contribute to the development of celiac disease. Growing insight into the variable clinical and histopathological presentation features of this disease has opened new perspectives for future research. A strict life-long gluten-free diet is the only safe and efficient available treatment, yet it results in a social burden. Alternative treatment modalities focus on modification of dietary components, enzymatic degradation of gluten, inhibition of intestinal permeability and modulation of the immune response. A small group of patients with celiac disease (2-5%), however, fail to improve clinically and histologically upon elimination of dietary gluten. This complication is referred to as refractory celiac disease, and imposes a serious risk of developing a virtually lethal Enteropathy-Associated T-Cell Lymphoma.
Abdulbaqi Altoma - One of the best experts on this subject based on the ideXlab platform.
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auto sct in refractory celiac disease type ii patients unresponsive to cladribine therapy
Bone Marrow Transplantation, 2011Co-Authors: Greetje J Tack, Wieke H M Verbeek, Abdulbaqi Altoma, Marielle J Wondergem, A Schmittel, M V Machado, F Perri, Gert J Ossenkoppele, Peter C Huijgens, Marco W J SchreursAbstract:Autologous hematopoietic SCT (auto-SCT) has been effective therapy for refractory disease, in both malignancies and severe autoimmune diseases. It seems feasible and safe for refractory celiac disease (RCD) type II, although long-term results have not been evaluated yet. With current therapies, progression into Enteropathy-Associated T-Cell Lymphoma (EATL) occurs in 60-80% patients, with a high mortality rate. Therefore, it is important to evaluate new treatment strategies. Between March 2004 and February 2010, 18 RCD II patients were evaluated for auto-SCT preceded by conditioning with fludarabine and melphalan, as a consequence of unresponsiveness to cladribine therapy. Adverse events, survival rate, EATL development and change in clinical, histological and immunological course were monitored. Thirteen patients were transplanted successfully and followed up for >2 years, 4-year survival rate was 66%. Only one patient died because of transplant-related complications. The majority of patients showed an impressive clinical improvement and five a complete histological remission. In five patients, auto-SCT could not be performed; they all died with a median survival of 5.5 months. EATL was observed in one transplanted patient, only after 4 years of follow-up. Auto-SCT after conditioning with high-dose chemotherapy in RCD II patients unresponsive to cladribine therapy is feasible and seems promising.
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evaluation of cladribine treatment in refractory celiac disease type ii
World Journal of Gastroenterology, 2011Co-Authors: Greetje J Tack, Otto Visser, Wieke H M Verbeek, Abdulbaqi Altoma, Dirk J Kuik, Marco W J Schreurs, Chris J. J. MulderAbstract:AIM: To evaluate cladribine [2-chlorodeoxyadenosine (2-CdA)] therapy in refractory celiac disease (RCD) II. METHODS: An open-label cohort-study of RCD II patients treated with 2-CdA was performed between 2000 and 2010. Survival rate, enteropathy associated T-Cell Lymphoma (EATL) occurrence, clinical course, and histological and immunological response rates were evaluated. RESULTS: Overall, 32 patients were included with a median follow-up of 31 mo. Eighteen patients responded well to 2-CdA. Patients responsive to 2-CdA had a statistically significant increased survival compared to those who were unresponsive. The overall 3- and 5-year survival was 83% in the responder and 63% and 22% in the non-responder group, respectively. The overall 2-year clinical, histological and immunological response rates were 81%, 47% and 41%, respectively. Progression into EATL was reported in 16%, all of these patients died. CONCLUSION: Treatment of RCD II with 2-CdA holds promise, showing excellent clinical and histological response rates, and probably less frequent transition into EATL.
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survival in refractory coeliac disease and enteropathy associated t cell Lymphoma retrospective evaluation of single centre experience
Gut, 2007Co-Authors: Abdulbaqi Altoma, Wieke H M Verbeek, M Hadithi, B M E Von Blomberg, C J J MulderAbstract:Background: Coeliac disease may be regarded as refractory disease (RCD) when symptoms persist or recur despite strict adherence to a gluten-free diet. RCD may be subdivided into types I and II with a phenotypically normal and aberrant intraepithelial T-Cell population, respectively. RCD I seems to respond well to azathioprine/prednisone therapy. RCD II is usually resistant to any known therapy and transition into Enteropathy-Associated T-Cell Lymphoma (EATL) is common. Aim: To provide further insight into RCD and the development of EATL, by reporting on long-term survival and risk of transition of RCD into EATL in a large cohort of patients with complicated coeliac disease. Design and Methods: Retrospective comparison of responses to therapy in four groups of patients with complicated coeliac disease: 43, RCD I; 50, RCD II (total), of whom 26 with RCD II developed EATL after a period of refractoriness to a gluten-free diet (secondary EATL) and 13 were EATL patients without preceding history of complicated coeliac disease (de novo EATL). Results: No coeliac-disease-related mortality was recognised in the RCD I group. The overall 5-year survival in the RCD I group it was 96%; in the RCD II (total) group was 58%; and in the RCD II group after developing EATL it was only 8%. The 2-year survival in the de novo EATL group was 20% versus 15% in secondary EATL group (p = 0.63). Twenty-eight (56%) of the 50 patients with RCD II died, 23 (46%) due to EATL, 4 due to a progressive refractory state with emaciation and 1 from neurocoeliac disease. Conclusion: Remarkably, no patient with RCD I developed RCD II or EATL within the mean follow-up period of 5 years (range 2–15 years). A total of 52% of the RCD II patients developed EATL within 4–6 years after the diagnosis of RCD II. More aggressive and targeted therapies seem necessary in RCD II and EATL.