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Peer-reviewedCC BY 4.0JACC Case Reports

Systemic Amyloidosis After Liver Transplantation From a Donor With Hereditary Transthyretin Amyloidosis

  • Moises Abtibol Machado1
  • Matheus Martins Monteiro2
  • Denison Clark Corrêa de Miranda1
  • Marcela Oliveira de Azevedo1
  • Rodrigo Fernandes de Castro1
  • Nayara do Carmo Santos Lima1
  • Diogo Corrêa Lamberti1
  • Andreza Araújo de Oliveira3
  • Kátia do Nascimento Couceiro1
  • João Marcos Bemfica Barbosa Ferreira1
  1. 1Universidade do Estado do Amazonas (UEA), Manaus, Amazonas, Brazil
  2. 2Hospital Beneficência Portuguesa de São Paulo, São Paulo, São Paulo, Brazil
  3. 3Universidade NiltonLins, Manaus, Amazonas, Brazil
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Abstract

Background: Acquired transthyretin (TTR) amyloidosis after domino liver transplantation is rare and may manifest years after the procedure, with neurologic involvement.

Case Summary: A 75-year-old man received a liver from a donor with TTR amyloid polyneuropathy. Twelve years later, he developed paresthesias, diarrhea, and blood pressure lability. Electroneuromyography showed a length-dependent, predominantly axonal sensorimotor polyneuropathy, with absent sympathetic skin responses, indicating autonomic involvement. Abdominal fat pad biopsy was Congo red positive, and TTR genetic testing was negative. Cardiac involvement was absent. He was treated with pregabalin and fludrocortisone, and patisiran was indicated as disease-modifying therapy.

Discussion: The association of progressive neuropathy, dysautonomia, gastrointestinal manifestations, and domino transplantation favored acquired TTR amyloidosis over diabetic neuropathy. Reported cases show onset after 5-10 years, with neuropathy preceding cardiac disease.

Take-Home Message: Domino transplant recipients require long-term follow-up, even with negative genetic testing and no initial cardiomyopathy, to enable early diagnosis and monitoring of systemic progression.

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Cite

BibTeXRIS
BibTeX
@article{machado2026systemic,
  title = {{Systemic Amyloidosis After Liver Transplantation From a Donor With Hereditary Transthyretin Amyloidosis}},
  author = {Moises Abtibol Machado and Matheus Martins Monteiro and Denison Clark Corrêa de Miranda and Marcela Oliveira de Azevedo and Rodrigo Fernandes de Castro and Nayara do Carmo Santos Lima and Diogo Corrêa Lamberti and Andreza Araújo de Oliveira and Kátia do Nascimento Couceiro and João Marcos Bemfica Barbosa Ferreira},
  journal = {JACC Case Reports},
  year = {2026},
  month = oct,
  doi = {10.1016/j.jaccas.2026.110670},
  url = {https://doi.org/10.1016/j.jaccas.2026.110670},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Systemic Amyloidosis After Liver Transplantation From a Donor With Hereditary Transthyretin Amyloidosis
AU  - Moises Abtibol Machado
AU  - Matheus Martins Monteiro
AU  - Denison Clark Corrêa de Miranda
AU  - Marcela Oliveira de Azevedo
AU  - Rodrigo Fernandes de Castro
AU  - Nayara do Carmo Santos Lima
AU  - Diogo Corrêa Lamberti
AU  - Andreza Araújo de Oliveira
AU  - Kátia do Nascimento Couceiro
AU  - João Marcos Bemfica Barbosa Ferreira
PY  - 2026
DA  - 2026/10/01
JO  - JACC Case Reports
DO  - 10.1016/j.jaccas.2026.110670
UR  - https://doi.org/10.1016/j.jaccas.2026.110670
AB  - Background: Acquired transthyretin (TTR) amyloidosis after domino liver transplantation is rare and may manifest years after the procedure, with neurologic involvement.  Case Summary: A 75-year-old man received a liver from a donor with TTR amyloid polyneuropathy. Twelve years later, he developed paresthesias, diarrhea, and blood pressure lability. Electroneuromyography showed a length-dependent, predominantly axonal sensorimotor polyneuropathy, with absent sympathetic skin responses, indicating autonomic involvement. Abdominal fat pad biopsy was Congo red positive, and TTR genetic testing was negative. Cardiac involvement was absent. He was treated with pregabalin and fludrocortisone, and patisiran was indicated as disease-modifying therapy.  Discussion: The association of progressive neuropathy, dysautonomia, gastrointestinal manifestations, and domino transplantation favored acquired TTR amyloidosis over diabetic neuropathy. Reported cases show onset after 5-10 years, with neuropathy preceding cardiac disease.  Take-Home Message: Domino transplant recipients require long-term follow-up, even with negative genetic testing and no initial cardiomyopathy, to enable early diagnosis and monitoring of systemic progression.
SN  - 2666-0849
ER  -