Hepatic Amyloidosis Manifesting as Budd-Chiari Syndrome: An Unusual Presentation.

Amyloidosis is a rare infiltrative multisystemic disorder characterized by protein misfolding, leading to progressive organ failure. It can be either acquired or hereditary. Very few case reports regarding hepatic amyloidosis with Budd-Chiari syndrome have been reported up to date. We report the case of a 45-year-old man presenting with abdominal distension, pain in the abdomen, and jaundice. Through right hepatic vein cannulation, HVPG (hepatic venous pressure gradient) was found to be 10 mmHg. The liver biopsy revealed near-total replacement of hepatic parenchyma by amorphous congophilic deposits with obliteration of sinusoids. Hepatic amyloidosis with hepatic venous occlusion is a rare entity.