Enteropathy Associated T-Cell Lymphoma in a Young Patient with No Prior History of Celiac Disease

Abstract

Purpose: Enteropathy associated T cell lymphoma (EATL) is a malignancy of the small intestine typically affecting the jejunum. It is a rare form of high grade non-Hodgkin lymphoma arising from T cells in the intra-epithelial mucosa of the small bowel with an annual incidence of 0.5-1 per million people in Western countries. Atypical cell lines arise as a consequence of longstanding celiac disease in genetically susceptible individuals. EATL presents commonly in the 3rd or 4th decade of life with a peak incidence in the 6th decade of life. Presenting symptoms typically include abdominal pain, malaise, anorexia, weight loss, and diarrhea. Approximately 50% of patients require exploratory laparotomy for complications of perforation, hemorrhage, or obstruction. A 27-year-old female presents with intermittent epigastric abdominal pain, nausea, vomiting, diarrhea, and weakness. Her past medical history was notable for sickle cell trait and iron deficiency anemia. Initial CT imaging revealed circumferential duodenal thickening. An initial endoscopy with mucosal biopsy revealed only nonspecific inflammatory changes. She was started on a proton pump inhibitor. Serologic workup for celiac disease was negative. Serologic testing for HTLV-1 was also negative. A repeat EGD performed 2 months later due to persistent symptoms showed diffuse ulceration starting in the second portion of the duodenum with mild circumferential narrowing. Biopsies were consistent with EATL. One month later she presented with peritonitis and suspected small bowel perforation requiring small bowel resection. Surgical specimens confirmed EATL. Her postoperative course was unremarkable until several days after initiating chemotherapy. She developed septic shock and hypoxemic respiratory failure requiring intubation. Her abdomen became increasingly distended. After a prolonged course in the ICU, the patient ultimately died. The suspicion of EATL warrants an extensive work-up including endoscopy, CT/PET, and capsule endoscopy. If an initial biopsy is negative, the patient should proceed with full thickness biopsy. If the clinical suspicion remains high, small bowel resection for pathologic examination should be considered. The diagnosis of EATL is typically ominous. The 5 year survival rate is 10-20%, mostly due to patients having advanced disease and severe malnutrition at the time of diagnosis. Treatment consists of chemotherapy (usually anthracyline based) and possibly hematopoietic stem cell transplant. Surgery is indicated only for complications of therapy such as obstruction or perforation. As EATL is a histologically high grade lymphoma, early diagnosis and treatment is crucial to overall survival.