Transmission of Systemic Transthyretin Amyloidosis by Means of Domino Liver Transplantation

Abstract

Familial amyloidotic polyneuropathy is a fatal autosomal dominant disease caused by amyloidogenic genetic variants of transthyretin. The liver is the predominant source of circulating transthyretin, and liver transplantation is the only treatment available for the disease.¹ Livers explanted from patients with familial amyloidotic polyneuropathy contain only microscopic amyloid deposits in hilar vessels and nerves and are otherwise uninvolved. Since 1995, more than 300 such livers removed at transplantation have been used sequentially as donor grafts for recipients with liver cancer or end-stage liver disease, in so-called domino liver transplantation.² We report here a case of systemic transthyretin amyloidosis in a 55-year-old man who had received a liver graft eight years earlier by means of domino transplantation from a 32-year-old patient with familial amyloidotic polyneuropathy who had the Val30Met variant of transthyretin.