Published online Jan 28, 2017. doi: 10.4254/wjh.v9.i3.126
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
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.
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.