Liver transplantation in familial polyamyloidosis: Case report

Abstract

Purpose: To report hepatic transplant, an established therapy for Familial Amyloidotic Polyneuropathy phenotypes, with good results and improved prognosis, as a successful strategy to treat patients with Familial Amyloid Cardiomyopathy, prompting careful analysis of patients with familial amyloidosis as candidates for this therapy, regardless the phenotype. Methods: A liver transplant was performed in a patient with atypical presentation of transthyretin amyloidosis, with severe neurologic and mild cardiac symptoms. The graft was taken from a deceased donor. Results: One year after transplantation, the patient had improved clinical characteristics, with cardiac and neurological stabilization. Conclusions: This case shows that a liver transplant could be efficient to treat patients with transthyretin amyloidosis with neurological and cardilogic features. The literature is consistent in the selection of patients with Peripheral neuropathy for transplantation, but it is not in patient with cardiologic features. This report shows that the transplantation should be considered upon complexes clinical features.