Enteropathy-type T-cell lymphoma (ETTL) is a rare disease with a poor prognosis. According to the World Health Organization (WHO) classification, it is a subtype of the peripheral T-cell lymphomas. This disease is associated with gluten-sensitive enteropathy, has a high risk of intestinal perforation and obstruction, and is refractory to chemotherapeutic treatment. We report the case of a 73-year-old woman who was diagnosed with enteropathy-type T-cell lymphoma of the small intestine, which was positive for the markers of cytotoxic T cells, CD3, CD8, and CD56, on immunohistochemical staining after resection of the perforated terminal ileum.
Aged ; Celiac Disease/*complications/pathology ; Female ; Gastrointestinal Neoplasms/*complications/pathology ; Humans ; Intestinal Perforation/*diagnosis/*etiology/pathology ; Lymphoma, T-Cell/*complications/pathology

Enteropathy-associated T-cell lymphoma (EATL) is an unusual primary gastrointestinal lymphoma, particularly associated with celiac sprue. This tumor usually affects the jejunum and grossly presents as multiple circumferential ulcers without the formation of definite tumor masses. Moreover, mesenteric lymph nodes are commonly involved. The patients have typically suffered from abdominal pain, diarrhea, or weight loss whereas some patients may manifest with nonspecific symptoms for a period of years or an acute emergency of perforation, obstruction, or hemorrhage. The clinical course of EATL is very unfavorable and the prognosis is poor. Both celiac sprue and EATL are very rare diseases in Asia, except India and Middle East. We report a 60-year-old male diagnosed as having EATL after segmental small bowel resection, who presented with recurrent gastrointestinal bleeding.
Celiac Disease/*complications ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Intestinal Neoplasms/complications/*diagnosis/pathology ; Intestine, Small/pathology ; Lymphoma, T-Cell/complications/*diagnosis/pathology ; Male ; Middle Aged ; Recurrence

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

Celiac disease is a chronic absorptive disorder of the small intestine caused by gluten. The prevalence rate of celiac disease is 1% in Western countries. But, it is rare in Asian countries, and there is no celiac disease reported in Korea. Here, we report a case of celiac disease. An 36-years-old woman complained non-specific abdominal pain and diarrhea. She had anemia and was taking medication for osteoporosis. Colonoscopy showed no abnormality except shallow ulcer at the terminal ileum. Gastroduodenoscopy showed micronodularity at the duodenum 2nd and 3rd portion. Capsule endoscopy and enteroscopy showed villous atrophy and blunting of villi from the duodenum. Small intestinal pathology showed villous atrophy with lymphocyte infiltration. After gluten free diet, diarrhea, abdominal pain, anemia and osteoporosis were improved. And, she felt well-being sensation. This is a first case of celiac disease in Korea.
Abdominal Pain/etiology ; Adult ; Anemia/etiology ; Capsule Endoscopy ; Celiac Disease/complications/*diagnosis/diet therapy/pathology ; Diarrhea/etiology ; Diet, Gluten-Free ; Duodenum/pathology ; Endoscopy, Gastrointestinal ; Female ; Humans ; Ileum/pathology ; Intestinal Mucosa/pathology ; Osteoporosis/etiology ; Tomography, X-Ray Computed ; Treatment Outcome

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

BACKGROUNDIntestinal T-cell lymphoma (ITCL) is a heterogeneous lymphoid neoplastic group with variable clinical and pathological features. ITCL in oriental countries is different from enteropathy-type intestinal T-cell lymphoma (ETCL) in relation to celiac disease and Epstein-Barr virus (EBV). The objective of this study was to investigate the clinicopathological features, immunophenotype, expression of cytotoxic molecule (TIA-1), T-cell receptor (TCR)-gamma gene rearrangement, and Epstein-Barr virus (EBV) latent infection in primary ITCL without celiac disease in Chinese.

METHODSThe clinical data of 42 patients were analyzed, and the patients were followed up. Compared with human reactive lymphoid tissues, in situ hybridization for EBER1/2, polymerase chain reaction for TCR-gamma gene rearrangement, and immunohistochemical staining for immunophenotypes, TIA-1 and EBV latent membrane proteins (LMP-1) were investigated. Survival curves of different clinicopathological features, immuno-phenotypes, expression of LMP1, TCR-gamma gene rearrangement and therapy were analyzed.

RESULTSThree fourths of the patients suffered from ITCL in China were men with a peak age incidence in the 4th decade. Common presenting features included fever and hemotochezia. The prognosis was poor with a median survival of 3.0 months. The lesions were mostly localized in the ileocecum and colon. About 38/42 (90.5%) patients demonstrated pleomorphic medium-sized on large cells. Histological features of celiac disease were rarely seen. All 42 patients with ITCL revealed CD45RO positive. Neoplastic cells partially expressed T-cell differentiated antigens (CD3epsilon, CD4, CD8) and NK cell associated antigen (CD56). The positive frequency of CD3epsilon, CD4, CD8 and CD56 was 28/42 (66.7%), 7/42 (16.7%), 10/42 (23.8%) and 12/42 (28.6%) respectively. Thirty-nine cells (92.9%) expressed TIA-1, but none expressed CD20 and CD68. More than half of the patients (64.3%, 64.3% and 59.5%) revealed TCR-gamma gene rearrangement by three different TCR-gamma primers respectively. EBER1/2 was detected in 41 (97.6%) of the 42 patients. The expression frequency of LMP-1 was 38.1% (16/42).

CONCLUSIONSPrimary ITCL without celiac disease in Chinese is a special highly EBV-associated clinicopathological entity. There are few similarities in patients with celiac disease in western countries. A small proportion of primary ITCLs in Chinese and extranodal NK/T-cell lymphoma of nasal type belong to the same spectrum.

Adolescent ; Adult ; Celiac Disease ; complications ; Child ; Epstein-Barr Virus Infections ; complications ; Female ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Humans ; Immunophenotyping ; In Situ Hybridization ; Intestinal Neoplasms ; immunology ; pathology ; virology ; Lymphoma, T-Cell ; immunology ; pathology ; virology ; Male ; Middle Aged ; RNA, Viral ; genetics ; Viral Matrix Proteins ; genetics