Outcomes of patients with familial transthyretin amyloidosis after liver transplantation

Dipti Banerjee, Lindsey E. Roeker, Martha Grogan, Paul Swiecicki, John Poterucha, Julie Heimbach, Steve Zeldenrust, Morie Gertz, Brooks Sayre Edwards, Richard Daly, Kyle W. Klarich, Angela Dispenzieri

Research output: Contribution to journalArticle

6 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)246-250
Number of pages5
JournalProgress in Transplantation
Volume27
Issue number3
DOIs
StatePublished - 2017

Fingerprint

Familial Amyloidosis
Liver Transplantation
Transplants
Liver
Mutation
Survival
Kidney
Amyloidosis, Hereditary, Transthyretin-Related
Prealbumin
Peripheral Nervous System Diseases
Alkaline Phosphatase
Transplantation
Outcome Assessment (Health Care)

Keywords

  • Heart transplant recipient
  • Hemic and lymphatic diseases
  • Kidney transplant recipient
  • Liver transplant recipient
  • Pathological conditions
  • Signs and symptoms

ASJC Scopus subject areas

  • Medicine(all)
  • Transplantation

Cite this

Outcomes of patients with familial transthyretin amyloidosis after liver transplantation. / Banerjee, Dipti; Roeker, Lindsey E.; Grogan, Martha; Swiecicki, Paul; Poterucha, John; Heimbach, Julie; Zeldenrust, Steve; Gertz, Morie; Edwards, Brooks Sayre; Daly, Richard; Klarich, Kyle W.; Dispenzieri, Angela.

In: Progress in Transplantation, Vol. 27, No. 3, 2017, p. 246-250.

Research output: Contribution to journalArticle

Banerjee, D, Roeker, LE, Grogan, M, Swiecicki, P, Poterucha, J, Heimbach, J, Zeldenrust, S, Gertz, M, Edwards, BS, Daly, R, Klarich, KW & Dispenzieri, A 2017, 'Outcomes of patients with familial transthyretin amyloidosis after liver transplantation', Progress in Transplantation, vol. 27, no. 3, pp. 246-250. https://doi.org/10.1177/1526924817715463
Banerjee, Dipti ; Roeker, Lindsey E. ; Grogan, Martha ; Swiecicki, Paul ; Poterucha, John ; Heimbach, Julie ; Zeldenrust, Steve ; Gertz, Morie ; Edwards, Brooks Sayre ; Daly, Richard ; Klarich, Kyle W. ; Dispenzieri, Angela. / Outcomes of patients with familial transthyretin amyloidosis after liver transplantation. In: Progress in Transplantation. 2017 ; Vol. 27, No. 3. pp. 246-250.
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AU - Zeldenrust, Steve

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