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Prog Transplant. 2017 Sep;27(3):246-250. doi: 10.1177/1526924817715463. Epub 2017 Jul 4.

Outcomes of Patients With Familial Transthyretin Amyloidosis After Liver Transplantation.

Author information

1
1 Mayo Medical School, Mayo Clinic, Rochester, MN, USA.
2
2 Department of Internal Medicine, Brigham and Women's Hospital, Boston, MA, USA.
3
3 Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.
4
4 Division of Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA.
5
5 Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA.
6
6 Transplant Center, Mayo Clinic, Rochester, MN, USA.
7
7 Division of Hematology, Mayo Clinic, Rochester, MN, USA.

Abstract

BACKGROUND:

Familial transthyretin amyloidosis is a disease caused by misfolded transthyretin aggregates that can impair multiple organ systems. Liver transplantation is the first-line treatment for familial transthyretin amyloidosis.

RESEARCH QUESTION:

Our objective is to study outcomes and survival among patients with familial transthyretin amyloidosis after transplantation.

DESIGN:

All patients undergoing orthotopic liver transplant for familial transthyretin amyloidosis at Mayo Clinic between 1997 and 2012 were reviewed. Baseline clinical characteristics, organs transplanted, and posttransplant clinical course were assessed.

RESULTS:

Of the 40 patients, 7 patients had the V30M mutation and 33 had other mutations. Nineteen patients received liver only, 19 liver and heart, and 2 combined liver, heart, and kidney transplants. The 5-year overall survival was 85% for those receiving multiple organ transplant and 52% for those receiving liver transplant only ( P = .057). There was no difference in overall survival based on mutation (V30M vs other mutations), but survival was confounded by varied disease involvement and organs transplanted. Those who had early death (≤24 months from liver transplant) had a higher incidence of baseline peripheral neuropathy, autonomic neuropathy, lower modified BMI, and higher alkaline phosphatase.

DISCUSSION:

Outcomes of orthotopic liver transplant in familial transthyretin amyloidosis are variable due to heterogeneity in mutations and patient status at the time of transplant. Familial transthyretin amyloidosis can progress, despite liver transplantation. Patients receiving combined liver, heart/kidney transplant demonstrated improved survival compared to liver transplant alone.

KEYWORDS:

heart transplant recipient; hemic and lymphatic diseases; kidney transplant recipient; liver transplant recipient; pathological conditions; signs and symptoms

PMID:
29187090
DOI:
10.1177/1526924817715463

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