Rare presentation of primary (AL) amyloidosis as gastrointestinal hemorrhage without systemic involvement

We are reporting a rare case of a patient with primary(AL) amyloidosis presenting with an acute non-varicealupper gastrointestinal hemorrhage in the absence ofother systemic involvement. The case report involves a58-year-old woman with significant cardiac history andhereditary blood disorder who came in complaining ofabdominal pain and coffee-ground emesis for two days.Computed tomography(CT) scan of the abdomen andpelvis with contrast revealed segmental wall thickeningof the proximal jejunum with hyperdense, heterog-enous luminal content. Similar findings were evident inthe left lower small bowel region, suspicious for smallbowel hematoma and the possibility of intraluminalclots. Esophagogastroduodenoscopy performed postresuscitation showed punctate, erythematous lesionsthroughout the stomach as well as regions of smallbowel mucosa that appeared scalloped, ulcerated, andhemorrhaged on contact. Despite initial treatment for immunostain-positive focal cytomegalovirus gastritis, follow-up esophagogastroduodenoscopy after two months continued to demonstrate friable and irregular duodenal mucosa hinting at a different underlying etiol-ogy. Pathology reports from analyses of biopsy samples highlighted infiltration and expansion of the lamina pro-pria and submucosa. Subsequent staining with congo red/crystal violet and appropriate subtyping established the diagnosis of AL(kappa)-type amyloidosis. The sig-nificance of this case lies in the fact that our patient did not have the typically seen diagnostic systemic involvements-namely of heart and kidneys-usually seen in primary(AL) amyloidosis patients. It was the persis-tent endoscopic findings and biopsy results which gave clues to the physicians regarding the possibility of an abnormal protein-deposition entity.