Published online Feb 28, 2016. doi: 10.4254/wjh.v8.i6.340
Peer-review started: October 9, 2015
First decision: November 4, 2015
Revised: December 14, 2015
Accepted: January 27, 2016
Article in press: January 29, 2016
Published online: February 28, 2016
We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis.
Core tip: Amyloidosis is a pathological process that encompasses a spectrum of disease resulting from the extracellular deposition of fibrillar amyloid protein. It can involve any organ isolated or in conjunction with other organs and can do so in the form of a focal, tumour-like lesion, or an infiltrative process. Amyloidosis localized to the liver has been rarely described. This case represents a rare instance of primary hepatic amyloidosis without evidence of primary or secondary cause of amyloid deposit posing considerable diagnostic and therapeutic challenge for the clinicians.