Synonym(s): Cholestatic hepatic amyloidosis

Hepatic amyloidosis with intrahepatic cholestasis is a form of primary hepatic amyloidosis characterized by extensive amyloid liver deposition and severe intrahepatic cholestasis simulating bile duct obstruction. This condition is very rare, reported in less than 50 patients worldwide. It is estimated to affect approximately 5% of patients with liver amyloidosis. The main clinical signs include hepatomegaly (usually marked; found in 92% of patients), ascites (in 56% of the cases), cholestatic icterus, and a markedly elevated serum alkaline phosphatase and C-reactive protein level with normal transaminases. Lethargy, abdominal pain, chronic nausea and weight loss may be present for months before the onset of jaundice. Hyperbilirubinemia is rare and is considered as predictor of a poor prognosis. Portal hypertension may occur. Liver histology shows considerable amyloid periportal, intraportal, vascular, and parenchymal deposition causing severe cholestasis and atrophy of hepatocytes. The disease has a rapid downhill course. The etiology of primary amyloidosis is related to abnormal plasma cell production of antibodies. Diagnosis is established by needle liver biopsy, histology and immunohistochemistry. Extrahepatic cholestasis, hepatic malignancy, chronic liver disease related to a viral, drug, alcoholic or metabolic origin, connective tissue disorders, and direct liver involvement with myeloma should be excluded. To date, there is no specific treatment for this condition. Survival, with any treatment tried, is reported to be three or four months from the onset of jaundice.