ABSTRACT
A 65-year-old male with a 25-year history of chronic alcoholism presented to the emergency department for a two-week history of non-radiating right upper quadrant abdominal pain associated with pruritus, nausea, coffee-ground emesis, and clay-colored stools. The exam was notable for icteric sclera, right upper quadrant abdominal tenderness, ascites, and hepatomegaly. Initial workup revealed new-onset unexplained elevated liver enzyme. The CT scan revealed diffuse liver cirrhosis, periportal lymphadenopathy, and stigmata of portal hypertension including hepatosplenomegaly, ascites, and varices. Esophagogastroduodenoscopy (EGD) with endoscopic ultrasound was performed, which showed gastritis and enlarged porta hepatis, which was ultimately biopsied and revealed extracellular amyloid deposition in peri-sinusoidal spaces consistent with amyloidosis. Transesophageal echocardiogram raised suspicion for cardiac involvement with amyloid deposit. The patient was started on steroids and chemotherapy with daratumumab, however his condition was complicated by septic shock, which led to an admission in the ICU followed by endotracheal intubation and multi-organ failure and eventual palliative care. Our case highlights the importance of clinical suspicion of GI amyloidosis in patients with constitutional symptoms including fatigue, weight loss, and unexplained liver disease. Once amyloidosis is considered, the diagnosis can be obtained by tissue biopsy from either the GI tract or subcutaneous adipose tissue.
