• EATL
• MEITL
• T-cell lymphoma
• duodenum
• lymphoma

• celiac disease
• lymphoma
• transplantation

• Carcinogenesis
• Coeliac disease
• Gastroenterology
• Oncology
• Small intestine cancer

• Male
• Humans
• Celiac Disease/complications
• Celiac Disease/diagnosis
• Enteropathy-Associated T-Cell Lymphoma/diagnosis
• Enteropathy-Associated T-Cell Lymphoma/complications
• Intestinal Diseases/complications
• Lymphoma, Non-Hodgkin/complications
• Abdominal Pain/complications

• EATL
• ITCL
• ITCLDGT
• MEITL
• celiac disease
• lymphoproliferative disorders
• small bowel lymphoma

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and rapidly progressive intestinal T-cell non-Hodgkin lymphoma associated with a very poor prognosis and a median survival of 7 mo. Advances in the identification of MEITL over the last two decades have led to its recognition as a separate entity. MEITL patients, predominantly male, typically present with vague and nonspecific symptoms and diagnosis is predominantly confirmed at laparotomy. Currently, there are no standardized treatment protocols, and the optimal therapy remains unclear.

We report a case of MEITL that was initially considered to be gastrointestinal stromal tumor (GIST) and Imatinib was administered for one cycle. The 62-year-old man presented with abdominal pain, abdominal distension, and weight loss of 20 pounds. Within 2 wk, the size of the mass considerably increased on computed tomography scans. The patient underwent surgery followed by chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) and stem-cell transplant. A correct diagnosis of MEITL was established based on postoperative pathology. Immunophenotypically, the neoplastic cells fulfilled the diagnostic criteria for MEITL as they were CD3⁺, CD4⁺, CD8⁺, CD56⁺, and TIA-1⁺.

Given that MEITL has no predisposing factor and presents with vague symptoms with rapid progression, the concomitant presence of abdominal symptoms and B symptoms (weight loss, fever, and night sweats) with hypoalbuminemia, anemia, low lymphocytic count and endoscopic findings of diffuse infiltrating type lesions should alert physicians to this rare disease, especially when it comes to Asian patients. Immediate laparotomy should then be carried out followed by chemotherapy and stem-cell transplant.

• Monomorphic epitheliotropic intestinal T-cell lymphoma
• Gastrointestinal stromal tumor
• Immunophenotypically
• Chemotherapy
• Stem-cell transplant
• Case report

• T lymphocytes
• autoimmunity
• coeliac disease
• gluten sensitive enteropathy
• lymphoma

• Marie Sklodowska-Curie Individual fellowship
• INSERM
• Foundation Princesse Grace
• Université Paris-Cité
• Association Française Des Intolérants au Gluten (AFDIAG)
• Foundation ARC-Recherche Clinique
• Investissement d’Avenir
• ANR
• Cancéropôle and INCa
• Inserm-Plan Cancer

• Celiac Disease
• Gastroenterology
• Humans
• Metabolic Diseases

It is challenging for pathologists to diagnose primary gastrointestinal T-cell neoplasms. Besides the rarity of the diseases, the small biopsy material makes it more difficult to differentiate between non-neoplastic inflammation and secondary involvement of extra gastrointestinal lymphoma. Since this group of diseases ranges from aggressive ones with a very poor prognosis to indolent ones that require caution to avoid overtreatment, the impact of the diagnosis on the patient is enormous. Although early treatment of aggressive lymphoma is essential, the treatment strategy is not well established, which is a problem for clinicians. This review provides a cross-sectional comparison of histological findings. Unlike previous reviews, we summarized up-to-date clinically relevant information including the treatment strategies as well as practical differential diagnosis based on thorough literature review.

Primary gastrointestinal (GI) T-cell neoplasms are extremely rare heterogeneous disease entities with distinct clinicopathologic features. Given the different prognoses of various disease subtypes, clinicians and pathologists must be aware of the key characteristics of these neoplasms, despite their rarity. The two most common aggressive primary GI T-cell lymphomas are enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma. In addition, extranodal natural killer (NK)/T-cell lymphoma of the nasal type and anaplastic large cell lymphoma may also occur in the GI tract or involve it secondarily. In the revised 4th World Health Organization classification, indolent T-cell lymphoproliferative disorder of the GI tract has been incorporated as a provisional entity. In this review, we summarize up-to-date clinicopathological features of these disease entities, including the molecular characteristics of primary GI T-cell lymphomas and indolent lymphoproliferative disorders. We focus on the latest treatment approaches, which have not been summarized in existing reviews. Further, we provide a comprehensive review of available literature to address the following questions: How can pathologists discriminate subtypes with different clinical prognoses? How can primary GI neoplasms be distinguished from secondary involvement? How can these neoplasms be distinguished from non-specific inflammatory changes at an early stage?

• EATL
• ITLPD-GI
• MEITL
• NK-cell enteropathy
• enteropathy-associated T-cell lymphoma
• indolent T-cell lymphoproliferative disorder
• monomorphic epitheliotropic intestinal T-cell lymphoma
• primary gastrointestinal T-cell lymphoma

• PCR for antigen receptor rearrangement
• dogs
• endoscopy
• enteropathy-associated T-cell lymphoma
• immunohistochemistry
• inflammatory bowel disease
• intestinal lymphoma

• carcinoma
• celiac disease
• complication
• lymphoma
• malignancy
• tumor

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare primary intestinal T-cell lymphoma, previously known as enteropathy-associated T-cell lymphoma type II. MEITL is an aggressive T-cell lymphoma with a poor prognosis and high mortality rate. The known major complications of MEITL are intestinal perforation and obstruction. Here, we present a case of MEITL that was diagnosed following upper gastrointestinal bleeding from an ulcerative duodenal lesion, with recurrence-free survival for 5 years.

A 68-year-old female was admitted to our hospital with melena and mild anemia. An urgent esophagogastroduodenoscopy (EGD) revealed bleeding from an ulcerative lesion in the transverse part of the duodenum, for which hemostatic treatment was performed. MEITL was diagnosed following repeated biopsies of the lesion, and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy was administered. She achieved complete remission after eight full cycles of CHOP therapy. At the last follow-up examination, EGD revealed a scarred ulcer and ¹⁸Fluorodeoxyglucose (¹⁸FDG) positron emission tomography/computed tomography showed no abnormal FDG accumulation. The patient has been in complete remission for 68 mo after initial diagnosis.

To rule out MEITL, it is important to carefully perform histological examination when bleeding from a duodenal ulcer is observed.

• Monomorphic epitheliotropic intestinal T-cell lymphoma
• Enteropathy-associated T-cell lymphoma type II
• Gastrointestinal bleeding
• Intestinal lymphoma
• Duodenal ulcer
• Case report

• Antineoplastic Combined Chemotherapy Protocols/therapeutic use
• Biopsy
• Cyclophosphamide/therapeutic use
• Doxorubicin/therapeutic use
• Enteropathy-Associated T-Cell Lymphoma
• Female
• Humans
• Lymphoma, T-Cell
• Melena/etiology
• Positron Emission Tomography Computed Tomography
• Vincristine/therapeutic use

• celiac disease
• gluten
• small bowel
• sprue

• Celiac disease
• EATL
• Intestinal lymphoma
• T cell Lymphoma

Enteropathy-associated T-cell lymphoma (EATL) is a rare invasive lymphoma derived from gastrointestinal epithelial T lymphocytes. EATL involving the whole gastrointestinal tract accompanied with early colon cancer is extremely rare.

We present the case of a 67-year-old man with diarrhea for more than 5 mo whose colonoscopy in another hospital showed multiple colonic polyps, which indicated moderate to severe dysplasia and focal early cancer. Therefore, he was referred to our hospital for further endoscopic treatment. Colonoscopy after admission showed that the mucosa of the terminal ileum and the entire colon were slightly swollen and finely granular. Endoscopic mucosal resection was performed for colonic polyps located in the liver flexure of the colon and descending colon, respectively. Histopathological findings revealed diffuse infiltration of medium-sized lymphoid cells in the colonic mucosa and visible lymphoepithelial lesions. The histopathology of the polyp in the descending colon indicated moderately differentiated adenocarcinoma limited to the mucosa with negative resection margins. Additionally, immunohistochemical analysis showed positive staining for CD7 and CD8. Therefore, we arrived at a diagnosis of EATL with early colon cancer. Subsequently, the patient was transferred to the hematology department for chemotherapy. The patient’s diarrhea was not significantly relieved after receiving chemotherapy, and he ultimately died of severe myelosuppression.

EATL should be considered in unexplained chronic diarrhea. EATL progresses rapidly with a poor prognosis, especially when accompanied with early colon cancer.

• Enteropathy-associated T-cell lymphoma
• Early colon cancer
• Endoscopic mucosal resection
• Case report
• T-cell lymphoma
• Diarrhea

• capsule endoscopy
• celiac disease
• enteroscopy
• gluten free diet
• refractory celiac disease

• HLA-DQ2
• HLA-DQ8
• T-cell lymphoma
• colorectal cancer
• gluten
• gluten-free diet
• refractory celiac disease
• small bowel adenocarcinoma

• Celiac Disease/complications
• Celiac Disease/diet therapy
• Diet, Gluten-Free
• Gastrointestinal Neoplasms/etiology
• Humans
• Lymphoproliferative Disorders/etiology
• Neoplasms/etiology

• lymphoma
• enteropathy-associated t cell lymphoma
• duodenum

• Adult
• Duodenal Diseases/pathology
• Enteropathy-Associated T-Cell Lymphoma/pathology
• Female
• Humans
• Intestinal Obstruction/complications
• Intestinal Perforation/etiology
• Intestinal Perforation/surgery
• Ovarian Neoplasms/secondary

Gastrointestinal (GI) lymphomas comprise a group of distinct clinicopathological entities of B- or T- cell type, with primary gastrointestinal Hodgkin lymphoma being extremely uncommon. The GI tract is the predominant site of extranodal non-Hodgkin lymphoma accounting for 30–40% of all extranodal lymphomas. In the Western world, the stomach is the most commonly involved site followed by the small bowel. Several chronic inflammatory and immune-mediated disorders which predispose to accelerated cell turnover may lead to the malignant transformation of gut lymphocytes and ultimately manifest as GI lymphoma. The challenge for the clinical gastroenterologist is that these tumors may have varied presentations, ranging from nonspecific symptoms such as dyspepsia or bloating to abdominal pain, nausea, vomiting, GI bleeding, diarrhea, weight loss or bowel obstruction.

We illustrate the range of presentations of GI lymphoma with examples based on consecutive cases evaluated at our institution over a 6-month period. These cases demonstrate how appropriately directed endoscopic evaluation with biopsies has the potential to provide a definitive diagnosis and allow the patient to proceed to definitive therapy.

The GI tract is the most commonly involved site for extranodal lymphoma with the stomach being most frequently involved organ. Chronic Helicobacter pylori infection, celiac disease, inflammatory bowel disease and autoimmune disorders may predispose to GI lymphoma. This heterogenous group of diseases has varied presentations that may mimic several other GI clinico-pathologic entities. GI lymphomas may be diagnosed with appropriately directed endoscopic evaluation coupled with generous tissue sampling and expert pathologic assessment. Management may range from antibiotic therapy, in the case of Helicobacter pylori-associated gastric MALT lymphoma, to chemotherapy with or without radiation and, in rare instances, surgery. There are presently no guidelines to direct endoscopic surveillance of GI lymphomas following treatment.

• gastric lymphoma
• gastrointestinal neoplasia
• gastrointestinal pathology

• antibody targeted therapy
• coeliac disease
• gastrointestinal lymphoma
• tumour markers

• Celiac disease
• Diagnostic work-up
• Histopathology
• Overall survival
• Small bowel adenocarcinoma
• Treatment options

• Autoimmune
• Celiac disease
• Gluten
• T cell
• Therapy

• Autoantibodies/immunology
• Celiac Disease/diagnosis
• Celiac Disease/therapy
• Diet, Gluten-Free
• GTP-Binding Proteins/immunology
• Genetic Predisposition to Disease
• Glutens/immunology
• HLA-DQ Antigens/genetics
• Humans
• Immune System/physiology
• Immune System/physiopathology
• Immunotherapy
• Intestinal Mucosa/immunology
• Intestinal Mucosa/pathology
• Protein Glutamine gamma Glutamyltransferase 2
• Risk Assessment
• Risk Factors
• Transglutaminases/immunology

Shiba dogs are predisposed to chronic enteropathy (CE) and have poorer prognosis than other dog breeds. The objective of this study was to investigate the significance of polymerase chain reaction for antigen receptor rearrangement (PARR) results on clinical findings and prognosis of Shiba dogs with CE. We retrospectively collected data on 22 Shiba dogs diagnosed as having CE. Fifty-nine percent of the dogs had clonality-positive results on PARR analysis. Furthermore, on histopathology, epitheliotropic behavior of small lymphocytes of the intestinal mucosa was observed significantly more frequently in dogs with clonal rearrangement of antigen receptor genes (P=0.027). The median overall survival time of clonality-positive dogs was 48 days (range, 4–239 days), compared to 271 days (range, 45–1,316+ days) in clonality-negative dogs. The median overall survival time of epitheliotropism-positive dogs was 76 days (range, 30–349 days) compared to 239 days (range, 4–1,316+ days) for epitheliotropism-negative dogs. Statistical analysis revealed that the clonality-positive result was associated with significantly shorter survival time (P=0.036). In contrast, presence or absence of epitheliotropism had no statistically significant effect on survival time (P=0.223). These cases might appropriately be diagnosed as small T-cell intestinal lymphoma; there are some common clinical and pathogenic features with human enteropathy-associated T-cell lymphoma type 2. The pathogenesis and poor prognosis for Shiba dogs with CE seem to be associated with this type of lymphoma, although further investigation is warranted.

• Shiba dogs
• chronic enteropathy
• dog
• lymphoma
• polymerase chain reaction for antigen receptor rearrangements

• Anemia
• Celiac disease
• Neuroendocrine tumor

• Hematopoietic stem cell transplantation
• Histology
• Molecular biology
• Natural killer/T-cell lymphoma
• Pathophysiology
• Peripheral T-cell lymphoma
• Treatment

• Adenocarcinoma
• Adenoma
• Carcinoid
• Crohn’s disease
• Familial adenomatous polyposis
• Lynch syndrome
• Peutz-Jeghers syndrome
• Small bowel tumors

• Humans
• Intestinal Neoplasms/diagnosis
• Intestinal Neoplasms/epidemiology
• Intestinal Neoplasms/therapy
• Intestinal Polyps/diagnosis
• Intestinal Polyps/epidemiology
• Intestinal Polyps/therapy
• Intestine, Small

Le lymphome T intestinal associé à une entéropathie ou Enteropathy associated T-cell lymphoma (EATL), est une complication rare de la maladie cœliaque (MC). Nous rapportons l'observation d'un lymphome T associée à une MC révélé par une occlusion intestinale aigue. Une patiente maghrébine de 38 ans, aux antécédents de stérilité et de douleurs abdominales chroniques, était admise en urgence pour occlusion intestinale aigue. L'intervention chirurgicale retrouvait une tumeur au dépend du grêle avec des adénopathies mésentériques. L'histologie et l'immunohistochimie de la pièce opératoire objectivait un lymphome T digestif CD3+ et le bilan immunologique de la maladie cœliaque était positif. Le diagnostic d'EATL était ainsi retenu. La patiente était mise sous chimiothérapie (CHOEP) et régime sans gluten avec une réponse complète au traitement. L'EATL est une complication rare de la MC qui peut être révélée par une occlusion intestinale. Son pronostic peut être amélioré par une prise en charge précoce associant chirurgie et chimiothérapie. Sa prévention passe par un diagnostic précoce de la MC et un régime sans gluten.

• Celiac disease
• EATL
• T-cell lymphoma
• bowel obstruction
• surgery