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.

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.