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Evaluation of Cladribine treatment in refractory celiac disease type Ⅱ

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World Journal of Gastroenterology 2011年第4期17卷 506-513页

作者： Greetje J Tack Wieke HM Verbeek Abdul Al-Toma Dirk J Kuik Marco WJ Schreurs Otto Visser Chris JJ Mulder Department of Gastroenterology and Hepatology VU University Medical Center Department of Gastroenterology and Hepatology St. Antonius Hospital Department of Epidemiology and Biostatistics VU University Medical Center Department of Pathology VU University Medical Center Department of Haematology VU University Medical Center

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.

关键词： Cladribine refractory celiac disease Clinical course Enteropathy associated T-cell lymphoma Survival

Possible relationship between refractory celiac disease and malignancies

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World Journal of Clinical Oncology 2022年第3期13卷 200-208页

作者： Kaan Demiroren Department of Pediatric Gastroenterology University of Health SciencesYuksek Ihtisas Teaching HospitalBursa 16000Turkey

celiac disease(CeD)is a chronic autoimmune disorder that is triggered by gluten in genetically susceptible individuals,and that is characterized by CeD-specific antibodies,HLA-DQ2 and/or HLA-DQ8 haplotypes,enteropathy and different clinical pictures related to many *** lymphoma may develop as a result of refractory *** a patient diagnosed with CeD is symptomatic despite a strict gluten-free diet for at least 12 months,and does not improve with severe villous atrophy,refractory CeD can be considered *** second of the two types of refractory CeD has abnormal monoclonal intraepithelial lymphocytes and can be considered as pre-lymphoma,and the next picture that will emerge is enteropathy-associated T-cell *** manuscript addresses"CeD and malignancies"through a review of current literature and guidelines.

关键词： refractory celiac disease Enteropathy-associated T-cell lymphoma Prelymphoma Low grade lymphoma

Hematopoietic stem cell transplantation for non-malignant gastrointestinal diseases

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World Journal of Gastroenterology 2014年第46期20卷 17368-17375页

作者： Abdulbaqi Al-toma Petula Nijeboer Gerd Bouma Otto Visser Chris JJ Mulder Department of Internal Medicine and Gastroenterology 3430 EM NieuwegeinThe Netherlands Department of Gastroenterology VU University Medical Centre1005 MB AmsterdamThe Netherlands Department of Hematology VU University Medical Centre1005 MB AmsterdamThe Netherlands

Both, autologous and allogeneic hematopoietic stem cell transplantation (HSCT) can be used to cure or ameliorate a variety of malignant and non-malignant diseases. The rationale behind this strategy is based on the concept of immunoablation using high-dose chemotherapy, with subsequent regeneration of naive T-lymphocytes derived from reinfused hematopoietic progenitor cells. In addition, the use of HSCT allows for the administration of high-dose chemotherapy (whether or not combined with immunomodulating agents such as antithymocyte globulin) resulting in a prompt remission in therapy-refractory patients. This review gives an update of the major areas of successful uses of HSCT in non-malignant gastrointestinal disorders. A Medline search has been conducted and all relevant published data were analyzed. HSCT has been proved successful in treating refractory Crohn’s disease (CD). Patients with refractory celiac disease type II and a high risk of developing enteropathy associated T-cell lymphoma have shown promising improvement. Data concerning HSCT and mesenchymal SCT in end-stage chronic liver diseases are encouraging. In refractory autoimmune gastrointestinal diseases high-dose chemotherapy followed by HSCT seems feasible and safe and might result in long-term improvement of disease activity. Mesenchymal SCT for a selected group of CD is promising and may represent a significant therapeutic alternative in treating fistulas in CD.

关键词： Hematopoietic stem cell transplantation Mesenchymal stem cells Non-malignant gastrointestinal diseases celiac disease refractory celiac disease Lymphoma Crohn’ s Ulcerative colitis Cirrhosis

celiac disease:Management of persistent symptoms in patients on a gluten-free diet

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World Journal of Gastroenterology 2012年第12期18卷 1348-1356页

作者： David H Dewar Suzanne C Donnelly Simon D McLaughlin Matthew W Johnson H Julia Ellis Paul J Ciclitira King's College London Division of Diabetes and Nutritional SciencesDepartment of GastroenterologyThe Rayne InstituteSt Thomas' HospitalLondon SE1 7EHUnited Kingdom

AIM:To investigate all patients referred to our center with non-responsive celiac disease (NRCD),to establish a cause for their continued ***:We assessed all patients referred to our center with non-responsive celiac disease over an 18-mo *** individuals were investigated to establish the eitiology of their continued *** patients were first seen in clinic where a thorough history and examination were performed with routine blood work including tissue transglutaminase antibody *** were also referred to a specialist gastroenterology dietician to try to identift any lapses in the diet and sources of hidden gluten ingestion.A repeat small intestinal biopsy was also performed and compared to biopsies from the referring hospital where ***,lactulose hydrogen breath testing,pancreolauryl testing and computed tomography scan of the abdomen were undertaken if the symptoms *** clinical progress was followed over a minimum of 2 ***:One hundred and twelve consecutive patients were referred with *** were found not to have celiac disease (CD).Of the remaining 100 patients,45% were not adequately adhering to a strict gluten-free diet,with 24 (53%) found to be inadvertently ingesting gluten,and 21 (47%) admitting *** colitis was diagnosed in 12% and small bowel bacterial overgrowth in 9%.refractory CD was diagnosed in 9%.Three of these were diagnosed with intestinal *** 2 years,78 patients remained well,eight had continuing symptoms,and four had ***:In individuals with NRCD,a remediable cause can be found in 90%:with continued gluten ingestion as the leading *** propose an algorithm for investigation.

关键词： celiac disease Non-responsive celiac disea-se refractory celiac disease Gluten Gluten-free diet