S5968 Systemic Light Chain Amyloidosis Presenting as Chronic Liver Disease

Introduction: Systemic amyloidosis is frequently under diagnosed as it presents with nonspecific symptoms and can affect any organ system. This case report discusses the unusual case of an asymptomatic 52-year-old man who was discovered to have elevated liver enzymes and subsequently diagnosed with AL amyloidosis as discovered by a liver biopsy. Case Description/Methods: A 52-year-old man with a history of coronary artery disease presented to the ED for evaluation of persistent jaundice and elevated transaminases. A right upper quadrant ultrasound was ordered which demonstrated hepatomegaly with steatosis and concern for chronic liver disease (CLD). The patient was discharged home with follow-up with his primary care provider. At his follow-up, his liver function tests had further increased: aspartate aminotransferase 197, alanine aminotransferase 132, Alkaline phosphatase 1603, total bilirubin 4.1, and gamma-glutamyl transferase 2023. The patient returned to the ED and underwent further workup, including further labs and imaging. A CT abdomen/pelvis demonstrated a decompressed gallbladder with reactive gallbladder wall edema and no significant dilation of the intrahepatic biliary ducts. An magnetic resonance imaging of the abdomen demonstrated hepatosplenomegaly with no evidence of obstructive biliopathy. Further laboratory workup was unremarkable, including ANA, anti-smooth muscle antibody, anti-microsomal antibody, ceruloplasmin, alpha-1-antitrypsin, iron panel and hepatitis panel. Given the patient's unexplained elevated transaminases, a trans-jugular liver biopsy was performed. Results were remarkable for apple-green birefringence with Congo red staining. Pathology was significant for severe amyloidosis deposition present in vascular wall and hepatocyte parenchyma. Further workup was performed, including esophagogastroduodenoscopy and colonoscopy. Biopsies demonstrated amyloid deposition. The patient was diagnosed with systemic amyloidosis. The patient underwent a bone marrow biopsy which supported the diagnosis of AL amyloidosis. He was subsequently started on daratumumab for treatment. Discussion: This case focuses on the implications of amyloidosis on chronic liver disease. Patients with amyloidosis who present as chronic liver disease are frequently misdiagnosed and are delayed further care, as with our patient. Additionally, this patient was an unusual presentation of AL amyloidosis, as the patient was asymptomatic. This case reflects the importance of amyloidosis as a consideration in newly diagnosed CLD, especially in patients who do not have predisposing risk factors to CLD, such as obesity, alcohol use, or hepatitis.