S2167 Gastric Amyloidosis Presenting as Acute Upper Gastrointestinal Bleeding

Abstract

INTRODUCTION: Amyloidosis is characterized by extracellular tissue deposition of fibrils, composed of insoluble low molecular weight protein subunits. The type, location, and extent of fibril deposition generates variable clinical manifestations. Gastrointestinal (GI) amyloid is infrequent and its presentation may be suggestive of more prevalent etiologies. We aim to describe and discuss a case of acute upper GI bleeding secondary to gastrointestinal amyloid. CASE DESCRIPTION/METHODS: A 76-year old male with coronary artery disease, hypertension, and diverticulosis presented to the emergency department with frank hematemesis, melena, and decreased level of consciousness. Management required ICU admission with transfusion, intubation, and hemodynamic support. Upper endoscopy was concerning for the presence of isolated gastric varices, and well as gastritis with erosions in the cardia and antrum. There were no esophageal varices. CT angiography was performed, with embolization of the left gastric artery. This study did not suggest portal hypertension, cirrhosis, or splenic vein thrombosis. “Fullness” seen at the cardia was not secondary to gastric varices. Subsequently, the patient developed bilateral pleural effusions and subsegmental pulmonary embolism with left atrial appendage thrombus, treated by thoracentesis and anticoagulation. Pleural fluid was negative for malignancy. Repeated gastroscopy with biopsies of the cardia were reported as active chronic gastritis and ulceration. However, clinical suspicion for malignancy remained high. DISCUSSION: Pathology was reported as chronic gastritis until case discussion between treating gastroenterologist and pathologist prompted staining for the presence of an infiltrative process. Biopsies from repeat gastroscopy were stained with Congo red, revealing submucosal amyloid deposits. Hematology was consulted and subsequent investigations demonstrated plasma cell dyscrasia and light-chain amyloidosis. Patient was started on protocol for multiple myeloma in January 2020. Unfortunately, the patient developed symptoms of cardiac amyloid, which was strongly suggested by echocardiogram and cardiac markers. There was progressive functional decline and inability to tolerate further chemotherapy. The case illustrates an acute presentation of upper GI amyloid with upper GI bleed. Recognition of the possible pathology is imperative for a timely diagnosis.