An Unusual Cause of Fulminant Liver Failure

Abstract

Purpose: Amyloidosis is a disorder of fibrillary protein deposition in various organ systems and it may be primary (AL) or secondary (AA). Primary amyloidosis can occur alone or in association with plasma cell dyscrasias such as multiple myeloma. Involvement of the liver has been reported in up to 70% of cases with AL amyloidosis. Fulminant liver failure is characterized by rapid development of hepatocellular dysfunction and encephalopathy in a patient with no prior history of liver disease. We report a case of fulminant liver failure due to amyloidosis in the setting of multiple myeloma. A 66-year-old white male with a 50-pack-year history of smoking presented with shortness of breath, atrial fibrillation, and hypotension. His past medical history consisted of hypertension, hyperlipidemia and benign prostatic hypertrophy. Two months prior he had been told of a mild elevation in his liver enzymes. He had not started any new medications in the past six months. His shortness of breath was due to new onset atrial fibrillation. His atrial fibrillation was controlled but he developed worsening hypotension and renal failure. The patient's hypotension did not improve with fluids and required intravenous vasopressors. He developed bilateral pleural effusion, anasarca and congestive heart failure with an EF of 60%. He slowly started developing hepatic failure and was worked up for secondary causes of liver disease and no etiology was found. Upon transfer to our hospital he was frankly jaundiced, had mild asterixis, and small ascites. A right heart catheterization and MRI were consistent with restrictive cardiomyopathy. Lytic lesions were identified. A bone marrow aspirate showed moderate atypical plasmacytosis and focal amyloid deposition. Flow cytometry was compatible with multiple myeloma. A liver biopsy showed widespread amyloid deposition throughout the hepatic parenchyma. He was deemed not a liver transplant candidate or bone marrow transplant and family decided for hospice care. An autopsy showed severe amyloid deposition in the liver and heart along with left ventricular hypertrophy. There was no amyloid deposition in the kidneys or lungs. Hepatic failure as a consequence of amyloidosis and multiple myeloma is a rare condition with few cases reported. This case adds to the literature of acute liver failure caused by amyloidosis.