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World J Hepatol. Jan 28, 2017; 9(3): 126-130
Published online Jan 28, 2017. doi: 10.4254/wjh.v9.i3.126
Iatrogenic amyloid polyneuropathy after domino liver transplantation
Diana Mnatsakanova, Saša A Živković
Diana Mnatsakanova, Saša A Živković, Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, United States
Author contributions: Mnatsakanova D and Živković SA contributed equally to this work, generated the tables and wrote the manuscript.
Conflict-of-interest statement: The authors declare no conflicts of interest regarding this manuscript.
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: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Saša A Živković, MD, PhD, Department of Neurology, University of Pittsburgh Medical Center, 3471 Fifth Ave #810, Pittsburgh, PA 15213, United States. zivkovics@upmc.edu
Telephone: +1-412-6471706 Fax: +1-412-6478398
Received: August 30, 2016
Peer-review started: September 1, 2016
First decision: September 29, 2016
Revised: October 14, 2016
Accepted: December 7, 2016
Article in press: December 9, 2016
Published online: January 28, 2017
Processing time: 146 Days and 6.7 Hours
Abstract

Liver transplantation has been used in treatment of transthyretin amyloidosis, and some patients undergo domino liver transplantation (DLT) with explanted liver being transplanted to another patient with liver failure as the liver is otherwise usually functionally normal. Until end of 2015, there were 1154 DLT performed worldwide. DLT for transthyretin amyloidosis is associated with the risk of developing de novo systemic amyloidosis and amyloid neuropathy, and the risk may be greater with some non-Val30Met mutations. De novo amyloid neuropathy has been described in up to 23% of transplant recipients. Neuropathy may be preceded by asymptomatic amyloid deposition in various tissues and symptoms of neuropathy started after a median of 7 years following DLT (5.7 ± 3.2 years; range 2 mo to 10 years). Typical initial symptoms include neuropathic pain and sensory loss, while dysautonomia usually starts later. Progression of neuropathy may necessitate liver re-transplantation, and subsequent improvement of neuropathy has been reported in some patients. Explant allograft recipients need close monitoring for signs of systemic amyloidosis, neuropathy and dysautonomia as progressive symptoms may require re-transplantation.

Keywords: Transthyretin; Familial amyloid neuropathy; Domino liver transplantation; Systemic amyloidosis; Acquired amyloid neuropathy

Core tip: Domino liver transplantation (DLT) has been used in treatment of transthyretin amyloidosis, with explanted liver being transplanted to another patient with liver failure as the liver is otherwise usually functionally normal. Domino liver explant recipients are at risk of developing de novo systemic amyloidosis and amyloid neuropathy has been described in up to 23% of transplant recipients after a median of 7 years following DLT. Typical initial symptoms include neuropathic pain and sensory loss, while dysautonomia usually starts later. Explant allograft recipients need close monitoring for signs of systemic amyloidosis, neuropathy and dysautonomia as progressive symptoms may require re-transplantation.