Hepatic Amyloidosis - When Looks are Deceptive!

The liver can be involved in many systemic diseases, including infectious, immunologic, metabolic, vascular, infiltrative, and neoplastic diseases. Amyloidosis is a rare systemic disorder characterized by the deposition of insoluble, fibril-forming amyloid proteins in the extracellular space of various organs and it is more frequently observed in the gastrointestinal tract, heart and kidney. Liver can also be involved but often remain clinically silent with abnormal liver function tests and organ enlargement. The clinical and imaging features of the disease are often nonspecific and a tissue biopsy is often required. Hepatic involvement of the systemic amyloidosis is usually presented as diffuse infiltration and hepatomegaly, but very rarely may appear as unusual focal mass. Hepatic dysfunction is usually subclinical, however, in some patients, there is massive involvement of the liver could lead to acute liver failure, acute on chronic liver failure and decompensated cirrhosis of liver. We present the two rare cases of hepatic involvement of systemic amyloidosis. First case is of primary AL-systemic amyloidosis that presented as hepatic lesions, in the setting of plasma cell dyscrasia. Second case is of secondary AA-systemic amyloidosis that presented as decompensated chronic liver disease reported first time in literature which required liver transplantation.