Abstract

INTRODUCTION: Organ transplants are a novel treatment that can significantly improve the health of patients. Domino liver transplants (DLT) offer a way to increase the supply of livers that are in dire shortage. DLTs are unique because they provide an opportunity to use the original liver of liver transplant recipients as a graft for another patient. DLT is performed with patients with disorders such as amyloidosis, a condition where abnormal protein aggregates are produced and deposited in a variety of organs. It allows for the transplantation of a complete liver, that is anatomically and functionally normal besides the production of amyloid. Familial transthyretin amyloidosis (TTR) is a form of amyloidosis where transthyretin protein variants build-up commonly leading to liver and cardiac dysfunction. We present a case where a patient with TTR underwent a liver transplant and the explanted liver was donated to another patient with primary biliary cirrhosis, an autoimmune liver disease that damages intrahepatic bile ducts. CASE DESCRIPTION/METHODS: A 53-year-old female with a TTR type familial amyloidosis (transthyretin serine-84 mutation) undergoes a liver transplant. She has lost a father and 2 siblings to heart disease secondary to amyloidosis. She also had cardiac involvement and a previous carpal tunnel release with a biopsy positive for amyloid. Her original liver is used as the donor liver for a 74-year-old male patient with primary biliary cirrhosis. The recipient had previously developed esophageal varices and an increase in ascites. After the transplant, the donor experienced complications including graft-versus-host disease (GVHD), pain, nausea, vomiting, and diarrhea. GVHD was localized to a rash to her trunk and resolved with treatment. The recipient of the DLT liver had initial edema but is doing well now. Both patients are still doing well 2 years post-surgery. DISCUSSION: DLT can offer a solution for patients in need of a liver, but factors such as age must be considered. A liver transplant is curative of TTR and the removed liver can be considered for DLT for recipients who are not expected to outlive the symptoms of TTR. The transplant is curative because the liver is the source of the abnormal transthyretin protein, so both the donor and recipient should be monitored closely. We present a case of successful DLT, the donor also underwent a successful liver transplant that was curative for TTR. The recipient was an older patient who is not likely to develop symptoms of TTR.

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