Introduction: Amyloidosis is a rare disorder characterized by the extracellular deposition of an abnormal fibrillary protein which can be found in any organ system. In the gastrointestinal tract, the AA subtype is predominantly found in the small intestine. Furthermore, amyloidosis is commonly associated with underlying inflammatory states or malignancy.Here we present a unique case where the diagnosis of small bowel amyloid was made accidentally from biopsies on a routine upper endoscopy.
Case Description/Methods: This is a case of a seventy-seven-year-old African American male with a history of prostate cancer and recently diagnosed Large B-cell Lymphoma who initially presented with constipation and epigastric pain. Unfortunately, the patient failed conservative management with use of laxatives, proton pump inhibitor and dietary modifications. A subsequent upper endoscopy was performed and elicited a duodenum marked by diffuse erythema, linear erosions and ulcerations. Multiple biopsies were performed showing extracellular deposits of pale amorphous glassy material in the lamina propria and positive Congo Red staining consistent with Amyloidosis. The patient was then evaluated by an oncologist and begun on chemotherapy for his lymphoma. Over time the patient had improvement in his symptoms.
Discussion: Studies suggest that only 16% of patients with AA Amyloidosis had strict involvement within the GI tract. The reported gastrointestinal presentations include abdominal pain, change of bowel habits, altered gut motility, spontaneous bowel perforation, GI bleeding and malabsorption. In addition, studies have shown that 10% of lymphomas predispose patients to developing amyloidosis. Due to the vague clinical presentation, the diagnosis of gastrointestinal amyloidosis is often delayed. Cat scan may show small bowel thickening and dilatation with a “double halo” appearance. Endoscopic evaluation should be considered where biopsies can be obtained to aid in diagnosis. Management centers on treating the underlying inflammatory process or malignancy. Our patient had notable improvement in his symptoms with treatment of his lymphoma. Therefore, in individuals with underlying systemic inflammatory processes and vague refractory symptoms we recommend performing endoscopy with biopsies to rule out this rare disease.
Figure: Image A and B are endoscopic images of the second part of the Duodenum. Image C is of the bulb of the duodenum. There are pathognomic endoscopic findings of thickened mucosal folds and diffusely friable granular mucosa. There are also multiple characteristic yellowish-white polypoid protrusions(highlighted by arrows) which are thick deposits of amyloid. Image Amyloid A is of an H&E stain, it is notable for significant intracellular deposition causing crowding and alteration of normal mucosal structure. Image Congo Red is stained with Congo Red, confirmatory of Amyloid, it can be seen to involve nearly ever layer of the duodenum.
Disclosures: Neil Sondhi indicated no relevant financial relationships. Nishi Pandey indicated no relevant financial relationships. Vincent Ganapini indicated no relevant financial relationships. Kovil Ramasamy indicated no relevant financial relationships. Hammad Choudhry indicated no relevant financial relationships. Antonios Tsompanidis indicated no relevant financial relationships. John Dedousis indicated no relevant financial relationships. Damanjit Ghuman indicated no relevant financial relationships.
Neil Sondhi, MD1, Nishi Pandey, DO1, Vincent Ganapini, DO2, Kovil Ramasamy, MD2, Hammad Choudhry, MD2, Antonios Tsompanidis, DO2, John Dedousis, MD2, Damanjit Ghuman, MD2. P0971 - Caught "Congo" Red Handed: A Unique Case of Small Bowel Amyloidosis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.