S6206 Celiac Disease Causing Non-Hodgkin Lymphoma—A Rare Clinical Phenomenon

Introduction: Enteropathy T-cell Lymphoma (EATL), a rare form of non-Hodgkin lymphoma that arises in the gastrointestinal tract and is highly associated with celiac disease (CD). Early recognition and diagnosis of EATL is challenging, as it often presents with non-specific gastrointestinal (GI) symptoms. Here, we present the unusual case of EATL diagnosed endoscopically via retrograde double balloon enteroscopy after presenting for symptoms of fever, weight loss, and abdominal pain. Case Description/Methods: A 54-year-old woman with recent history of pulmonary embolism presented to the emergency department with fever and abdominal pain for approximately 3 months. On presentation, the patient was febrile to 101 F, but otherwise hemodynamically stable. Computed tomography showed small bowel intussusception, scattered mesenteric lymph nodes and thickened bowel without evidence of obstruction; subsequent computed tomography showed resolution of intussusception. She was given antibiotics and discharged home. One month later, she presented again due to persistent fevers and abdominal pain, as well as new shortness of breath. Labs showed leukocytosis, microcytic anemia, elevated INR, and hypoalbuminemia. She was also found to be coronavirus disease 2019 positive. Computed tomography of abdomen and pelvis angiogram showed multiple thromboembolic disease as well as thickening, edema, and mucosal hyper-enhancement of the small bowel. Additional labs were remarkable for elevated tissue transglutaminase IgA levels. She underwent esophagogastroduodenoscopy and colonoscopy with biopsies, however prep was poor. Duodenal biopsy confirmed the diagnosis of CD. She underwent retrograde double balloon enteroscopy for evaluation of the distal ileum which showed a large fungating mass with ulceration. A tissue biopsy confirmed a diagnosis of EATL. Shortly after hospital discharge, she was started on chemotherapy. Unfortunately, she had disease progression requiring salvage treatment. Her course was complicated by ifosfamide toxicity, bowel perforation, and septic shock ultimately resulting in her death 4 months after diagnosis. Discussion: EATL is an aggressive GI malignancy that is highly associated with CD. Prompt recognition is often overlooked due to non-specific presenting symptoms. This case highlights the importance of considering EATL in the evaluation of patients with CD and non-specific GI symptoms. Furthermore, this case demonstrates the utility of balloon-assisted enteroscopy in making the correct diagnosis prior to an intestinal complication requiring emergency surgery.