Síndromes linfoproliferativos gastrointestinales / Gastrointestinal lymphoproliferative syndrome

The gastrointestinal lymphoid system plays a relevant role. The daily and continuous interaction between gastrointestinal lymphocytes with food and intestinal microbes requires precise functioning. The pathologic spectrum of lymphocyte malfunction results in lymphomas. MALT lymphoma is the most frequently diagnosed lymphoma, but there are other lymphoproliferative diseases such as diffuse large B cell lymphoma, mantle cell lymphoma and T associated lymphoma. The gastroenterologist and the endoscopist need to know these diseases in detail to achieve early diagnosis and treatment.

El sistema linfoide de defensa abdominal tiene un relevante rol en el buen funcionamiento sistémico. La interacción diaria y continua con patógenos alimentarios y microbios comensales intestinales precisa un estrecho funcionamiento. Las alteraciones linfoides clonales favorecen el desarrollo de linfomas de diversos tipos. Si bien, el linfoma asociado a tejido linfoide de mucosas (MALT) es el más conocido en contexto de su asociación con Helicobacter pylori, el tracto gastrointestinal se puede ver afectado por otros linfomas como el linfoma difuso de células grandes B y linfomas indolentes como el linfoma folicular, el linfoma del manto y el linfoma T asociado a enteropatía. El gastroenterólogo y endoscopista precisan conocer en detalle estas entidades para un oportuno diagnóstico y adecuado tratamiento.

Endoscopic Findings of Enteropathy-Associated T-Cell Lymphoma Type II: A Case Series

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.

Linfoma de células T asociado con enteropatía tipo II / Type ii enteropathy-associated t-cell lymphoma

El linfoma de células T asociado con enteropatía tipo II es una rara variante del T periférico, el cual se puede ubicar en intestino delgado y presenta características inmunofenotípicas específicas. El presente estudio relata el caso de un hombre de 46 años sin antecedentes médicos que presentó perforación del intestino delgado con posterior diagnóstico por histopatología de un linfoma de células T asociado con enteropatía tipo II.

Type II enteropathy-associated T-cell lymphoma is a rare type of peripheral T-cell lymphoma that may involve the small intestine and has specific immunophenotypic features. This study reports the case of a 46-year old male with no history of previous illnesses who presented a small intestine perforation. A hispathologic diagnosis of type II enteropathy-associated T-cell lymphoma was made.

Primary gastrointestinal lymphomas - A study of 81 Cases from a Tertiary Healthcare Centre.

Purpose: Retrospective analysis of 81 routinely diagnosed gastrointestinal (GI) lymphoma to illustrate clinicopathological and immunohistochemical characteristics with predisposing condition. Materials and Methods: Age, sex, site, tumour stage, associated pathological features like lympho-epithelial lesion (LEL), atrophic gastritis (AG), intestinal metaplasia (IM) and enteropathy changes were analysed. Requisite immunohistochemical panel was applied wherever needed. Results: There were 55 male and 26 female patients with median age of 54.5 years. Site wise distributions were stomach 40, small intestine 22, colon 4, cecum 2, ileocecum 3, esophagus 1 and multiple sites 9. Histological subtypes were mucosa associated lymphoid tissue lymphoma (MALTOMA) 48, diffuse large B cell lymphoma (DLBL) 21, T cell lymphoma 9 [5 anaplastic large cell lymphoma (ALCL) and 4 enteropathy associated T cell lymphoma (EATL)], immunoproliferative small intestinal disease (IPSID) 2 and follicular lymphoma 1. LEL was present in 31 cases. Of the 19 AG, 8 had associated IM, and 1 case each had associated H Pylori infection and neuroendocrine tumor. Enteropathy was observed in 4 EATL, and one case each of DLBL and high grade MALTOMA. Giardia infection was present in 1 low grade duodenal MALTOMA. Of the 24 resected specimens, 16 were stage IE, 7 stage IIE and 1 stage IV (Mushoff's staging). Conclusion: Primary GI lymphoma was frequently observed in 6 th decade of life with male preponderance. Stomach was the commonest site and high grade MALTOMA being the commonest histological variant. Isolated colonic involvement and intestinal perforations were not infrequent. Rare variants like ALCL and follicular lymphomas were also observed.

A Case of Type II Enteropathy-Associated T-Cell Lymphoma with Epstein-Barr Virus Positivity

No abstract available.

Type II enteropathy-associated T-cell lymphoma: a clinicopathologic study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunohistochemical findings, differential diagnosis and prognosis of type II enteropathy-associated T-cell lymphoma (EATL).</p><p><b>METHODS</b>Fourteen 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.</p><p><b>RESULTS</b>There 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.</p><p><b>CONCLUSIONS</b>EATL 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.</p>

A Case of Enteropathy-Type T-Cell Lymphoma Diagnosed by Small Bowel Enteroscopy: A Perspective on Imaging-Enhanced Endoscopy

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.

Enfermedad Celiaca y Obstrucción Intestinal por Linfoma de Células T / Celiac disease and intestinal obstruction by T cell lymphoma

Paciente varón de 55 años, raza blanca (ascendencia española), natural y procedente de Ayacucho, que ingresó por una enfermedad de seis meses de evolución, caracterizada por dolor abdominal tipo cólico y baja ponderal de 10 Kg. Había estado hospitalizado seis meses antes y dado de alta con el diagnóstico de síndrome de intestino irritable. Entre sus antecedentes refería intolerancia a la lactosa y artritis reumatoidea. Los exámenes mostraron: Hb:12 g/dl, leucocitos: 5260 cel/mm, abastonados: 11%, albúmina: 2,7 mg/ml y VSG: 33mm/h. El resto de exámenes -incluyendo los marcadores tumorales- fueron normales. El tránsito gastrointestinal mostraba las asas delgadas con fragmentación del bario y dilatación focal moderada de un asa yeyunal distal. La tomografía de tórax, abdomen y pelvis sin alteraciones; Rx tórax normal. La colonoscopia fue normal; el íleon tenía pocos pliegues y pequeñas nodulaciones, las biopsias indicaron "ileitis inespecífica". La endoscopia mostró gastritis y una cicatriz de úlcera duodenal; la mucosa duodenal mostraba áreas con aspecto de mosaico rosado-blanquecino. La biopsia duodenal evidenció acortamiento de vellosidades, infiltrado inflamatorio crónico e incremento de linfocitos intraepiteliales, hallazgos compatibles con los criterios de celiaquía Marsh-tipo 3. Los anticuerpos IgA antiendomisio y antitransglutaminasa tisular estaban incrementados. Durante su hospitalización aumentó el dolor y aparecieron signos de obstrucción. En la laparotomía se encontraron una tumoración yeyunal estenosante y una perforación adyacente. El espécimen mostró un linfoma intestinal de células T. Se ha demostrado que existen más celiacos subclínicos que celiacos con esprue clásico; el conocimiento de esta situación nos debe llevar a tenerla presente por sus complicaciones o asociaciones, una de las cuales es el linfoma primario intestinal.

A male patient, 55 years old, born in Ayacucho, with Spanish ancestors, was hospitalized through emergency referring abdominal pain, and 10 kilograms weight loss. Six months before he was diagnosed as having irritable bowel syndrome. His previous diseases were rheumatoid arthritis and intolerance to lactose. Laboratory results were: Hb 12 gr./dL, white cells 5200 per mm3, albumin 2.7 gr./dL, erythrocyte sedimentation rate 32 mm/hr., and tumor markers were negative. Radiographic study of the small bowel showed barium fragmentation, and a focal dilation in distal jejunum. Chest X-ray and CT scan of thorax, abdomen and pelvis were normal. Colonoscopy was normal for colonic mucosa, but in ileum it showed an irregular mucosa, little nodules and fewer folds than usual. Biopsy from ileum demonstrated unspecific inflammation. Upper endoscopy showed gastritis, a duodenum scar ulcer and an irregular mosaic pattern pink and white. Duodenum biopsy demonstrated short villi, chronic inflammation and an increase in the number of intraepithelial lymphocytes, all these was consistent with celiac disease Marsh 3. Antibodies anti-endomisium and anti-transglutaminase were positive. After some days he developed signs of bowel obstruction and was operated. A tumor was found in jejunum with a bowel perforation. Pathological study showed a small bowel T-cell lymphoma. Fortunately this patient did well, and was sent home to continue treatment on ambulatory basis. Celiac disease is more common than what is thought, and it has been demonstrated that there are more persons with subclinical celiac disease, than those with the typical clinical pattern. It is necessary to be aware of this disease to improve diagnosis in order to avoid late complications as small bowel lymphoma.

A Case of Eneropathy-associated T-cell Lymphoma Presenting with Persistent Abdominal Pain and Diarrhea / 대한소화기내시경학회지

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.

A Case of Eneropathy-associated T-cell Lymphoma Presenting with Persistent Abdominal Pain and Diarrhea / 대한소화기내시경학회지

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.

A Case of Enteropathy-Associated T-cell Lymphoma (EATL) Presenting Perforation after Chemotherapy for Primary Intestinal Lymphoma / 대한소화기내시경학회지

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.