Hepatic amyloidosis: A rare entity

Abstract

Hepatic amyloidosis is a unique and uncommon disease which shows its presentation in the form of an infiltrative disease involving the liver. Amyloidosis is a systemic disease entity which is characterized by deposition of amyloid protein in the extracellular tissues of multiple organs. A 45-year-old female was admitted with complaints of yellowish discoloration of sclera and decreased appetite since 1 month. Liver enzymes were raised with a significantly high serum alkaline phosphatase. Microscopy showed linear sinusoidal deposition of an amorphous eosinophilic material which was also causing atrophy of liver cell plates. The portal tracts as well showed similar eosinophilic deposits. There was no significant lobular or portal inflammation. The material was found to be congophilic on congo red stain and it also showed apple green birefringence on polarizing microscopy. A diagnosis of hepatic amyloidosis was made. Amyloidosis of the liver is a rare disease entity that often presents with vague, nonspecific findings and has a poor prognosis. Prompt diagnosis and management are required to improve outcomes for this patient population. Further investigation and research are needed to elucidate the risks and effectiveness of hepatic amyloidosis treatment regimens. Liver involvement of amyloidosis presents with nonspecific clinical symptoms such as weight loss and fatigue. The prompt diagnosis is however very critical as liver-biopsy-proven amyloidosis has a poor prognosis as the median survival is only 8.5 months. Progressive organ involvement most often leads to organ malfunction and subsequently death due to renal and/or cardiac involvement.