Amyloidosis: An Act of Deception in the Liver

Abstract

We report a case of end-stage liver disease initially attributed to acute on chronic liver failure (ACLF) in a 59 YO African American female. She presented encephalopathic with stable vital signs. Her laboratory tests were as follows: bilirubin: 24.3 mg/dL, INR 2, Creatinine 4.2 mg/dL, MELD of 40 with rapidly declining renal function. Magnetic resonance cholangiopancreatography (MRCP) was negative. Her serologic work-up was unrevealing. She was deemed to have decompensated cryptogenic cirrhosis for which she received simultaneous liver & kidney transplantation. Her explanted liver revealed extensive amyloidosis, AL Amyloid kappa type. Urine electrophoresis showed a monoclonal free kappa light chain. Bone marrow biopsy and flow cytometry were negative for plasma cell dyscrasia. Further work up suggested no systemic involvement. We present this rare case of primary hepatic amyloidosis and discuss the importance of this diagnostic consideration in patients with otherwise unclear etiology of liver disease.