Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Feb 28, 2016; 8(6): 340-344
Published online Feb 28, 2016. doi: 10.4254/wjh.v8.i6.340
Primary hepatic amyloidosis: A case report and review of literature
Nikhil Sonthalia, Samit Jain, Sunil Pawar, Vinay Zanwar, Ravindra Surude, Praveen M Rathi
Nikhil Sonthalia, Samit Jain, Sunil Pawar, Vinay Zanwar, Ravindra Surude, Praveen M Rathi, Department of Gastroenterology, Topiwala National Medical College and BYL Ch Hospital, Mumbai 400008, Maharashtra, India
Author contributions: All authors contributed equally to the manuscript.
Institutional review board statement: The case report was exempt from the institutional review board standards at the BYL Nair Ch. Hospital and Topiwala National Medical College.
Informed consent statement: The patient involved in the study gave her written informed consent authorizing use and disclosure of her protected health information.
Conflict-of-interest statement: Authors have no conflict of interest to be declared.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Dr. Nikhil Sonthalia, Senior Resident, Department of Gastroenterology, Topiwala National Medical College and BYL Ch Hospital, Dr. A.L Nair Road, Mumbai 400008, Maharashtra, India.
Telephone: +91-900-4203165 Fax: +91-022-23016139
Received: October 1, 2015
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.

Keywords: Amyloidosis, Congo red staining, Isolated hepatic amyloidosis, Amyloid associated protein, Immunofixation electrophoresis

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.