No abstract available.
Enteropathy-Associated T-Cell Lymphoma* ; Herpesvirus 4, Human*

Enteropathy-type T-cell lymphoma (ETL) or enteropathy-associated T-cell lymphoma is a very rare malignant intestinal tumor. ETL is usually diagnosed by surgery. Endoscopic findings of ETL are not well known, and there are few reports of findings from endoscopy that has been performed only using white light. Additionally, there are no definite treatment guidelines for ETL. Therefore, we report a case of ETL diagnosed by enteroscopy with imaging-enhanced endoscopy and also review recently developed treatment options.
Endoscopy ; Enteropathy-Associated T-Cell Lymphoma ; Humans ; Light ; Lymphoma, T-Cell ; T-Lymphocytes

Enteropathy-associated T-cell lymphoma (EATL) is an unusual primary gastrointestinal lymphoma, and it is particularly associated with celiac sprue. These patients typically suffer from abdominal pain, diarrhea and/or weight loss. Primary intestinal T-cell lymphoma without celiac sprue is known to be rare. We report here on a case of EATL that presented with persistent abdominal pain and diarrhea, but this patient was without celiac sprue.
Abdominal Pain* ; Celiac Disease ; Diarrhea* ; Enteropathy-Associated T-Cell Lymphoma ; Humans ; Intestines ; Lymphoma ; Lymphoma, T-Cell* ; T-Lymphocytes* ; Weight Loss

Enteropathy-associated T-cell lymphoma (EATL) is an unusual primary gastrointestinal lymphoma, and it is particularly associated with celiac sprue. These patients typically suffer from abdominal pain, diarrhea and/or weight loss. Primary intestinal T-cell lymphoma without celiac sprue is known to be rare. We report here on a case of EATL that presented with persistent abdominal pain and diarrhea, but this patient was without celiac sprue.
Abdominal Pain* ; Celiac Disease ; Diarrhea* ; Enteropathy-Associated T-Cell Lymphoma ; Humans ; Intestines ; Lymphoma ; Lymphoma, T-Cell* ; T-Lymphocytes* ; Weight Loss

Enteropathy-Associated T-Cell Lymphoma ; classification ; pathology ; Gastrointestinal Tract ; pathology ; Humans

Intestinal T-cell lymphomas are fairly uncommon, and can sometimes be associated with enteropathy. Enteropathy-associated T-cell lymphoma (EATL) is commonly accompanied by a nonspecific mucosal ulceration, similar to that observed as a complication of celiac disease. The clinical course of EATL is quite unfavorable, and tends to have a generally poor prognosis. When a tumor invades the bowel wall and is treated with corticosteroids and chemotherapy, cell lysis with perforation often occurs, particularly in case of lymphoma. Recent data indicate that extensive resection may improve local control, and eliminate the risk of early mortality due to visceral perforation or hemorrhaging in unresected lesions during chemotherapy. Here, we report the case of a 51-year-old male who was diagnosed with primary gastrointestinal lymphoma after colonoscopy, and presented with EATL after emergent exploratory laparatomy. We also include a review of the literature regarding this uncommon entity.
Adrenal Cortex Hormones ; Celiac Disease ; Colonoscopy ; Drug Therapy* ; Enteropathy-Associated T-Cell Lymphoma* ; Gastrointestinal Hemorrhage ; Humans ; Lymphoma* ; Lymphoma, T-Cell ; Male ; Middle Aged ; Mortality ; Prognosis ; Ulcer

Enteropathy-associated T-cell lymphoma (EATL) is a rare extranodal T-cell lymphoma arising from the intestine. Two types of EATL have been reported. In contrast to the classic EATL type I, EATL type II occurs sporadically, is unrelated to celiac disease, and comprises 10% to 20% of all EATL cases. A total of five cases of EATL type II were diagnosed at our clinic from January 2009 to September 2012. Four of the five patients were diagnosed with the help of endoscopy. Among the four patients, two of the cases involved both the small and large intestines, whereas in the other two patients, EATL was limited to the small intestine. Common endoscopic findings included innumerable fine granularities (also called mosaic mucosal patterns) and diffuse thickening of the mucosa with a semicircular shallow ulceration in the lesions of the small bowel. In contrast, the endoscopic findings of the colon were nonspecific and could not distinguish EATL type II from other diseases. There are only few published reports regarding the representative endoscopic findings of EATL. Here, we present the clinical and endoscopic findings of four cases of EATL type II diagnosed by endoscopy.
Aged ; Celiac Disease/complications ; *Colonoscopy ; Enteropathy-Associated T-Cell Lymphoma/etiology/*pathology ; Female ; Humans ; Intestinal Mucosa/pathology ; Intestine, Large/pathology ; Intestine, Small/pathology ; Male ; Middle Aged

Antigens, CD ; metabolism ; CD3 Complex ; metabolism ; Celiac Disease ; complications ; Chromosome Aberrations ; Chromosomes, Human, Pair 9 ; Diagnosis, Differential ; Enteropathy-Associated T-Cell Lymphoma ; complications ; genetics ; metabolism ; pathology ; Humans ; Integrin alpha Chains ; metabolism ; Intestinal Mucosa ; pathology ; Ki-1 Antigen ; metabolism ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Lymphoma, Large B-Cell, Diffuse ; pathology

OBJECTIVETo study the clinicopathologic features, immunohistochemical findings, differential diagnosis and prognosis of type II enteropathy-associated T-cell lymphoma (EATL).

METHODSFourteen cases of type II EATL encountered in Department of Pathology, Nanjing General Hospital were retrospectively reviewed. The clinical data, histologic features, immunohistochemical findings and follow-up information were analyzed, with literature review.

RESULTSThere were altogether 12 males and 2 females. The median age of patient was 49 years. The sites of involvement included jejunum (10 cases) and ileum/colon (4 cases). The patients often presented with an abdominal mass, abdominal pain, diarrhea and constitutional symptoms such as fever, night sweating and cachexia. There was no clinical evidence of gluten-sensitive enteropathy. Histologically, the lymphoma cells showed full-thickness infiltration of the intestinal wall. They contained round hyperchromatic nuclei and pale cytoplasm. The stroma was minimally inflamed, with or without associated coagulative necrosis. A remarkable finding was the presence of villous atrophy, cryptal hyperplasia and intraepithelial lymphocytosis. Immunohistochemical study showed that the tumor cells expressed CD3, CD43 and CD8 (14/14). Some of them were also positive for CD56 (11/14) and CD30 (2/14). The staining for CD4, CD20, CD79a and myeloperoxidase was negative. A high proliferation index was demonstrated by Ki-67 immunostain. In-situ hybridization for EBER was negative. Follow-up data were available in 9 cases. The duration of follow-up ranged from 6 months to 36 months. Seven patients died within 14 months.

CONCLUSIONSEATL is a rare type of lymphoma with intestinal involvement. Associated enteropathy is not demonstrated, in contrast to cases encountered in Nordic countries. A correct diagnosis requires evaluation of clinical manifestations, pathologic features and ancillary study results.

Adolescent ; Adult ; Aged ; CD3 Complex ; metabolism ; CD8 Antigens ; metabolism ; Diagnosis, Differential ; Enteropathy-Associated T-Cell Lymphoma ; genetics ; immunology ; pathology ; surgery ; Female ; Follow-Up Studies ; Gene Rearrangement, T-Lymphocyte ; Humans ; Ileal Neoplasms ; genetics ; immunology ; pathology ; surgery ; Jejunal Neoplasms ; genetics ; immunology ; pathology ; surgery ; Leukosialin ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Young Adult