Acquired transthyretin amyloidosis after domino liver transplant

Phenotypic correlation, implication of liver retransplantation

Eli Muchtar, Martha Grogan, Surendra Dasari, Paul J. Kurtin, Morie Gertz

Research output: Contribution to journalArticle

Abstract

Reports of acquired ATTRm in domino liver transplant (DLT) recipients are infrequent and the approach to management is unknown. We describe two patients, an inherited ATTRm patient who underwent liver transplant and his liver recipient who developed acquired ATTRm 8 years following transplantation. The clinical manifestations of amyloidosis were similar between patients and consisted of peripheral neuropathy, diarrhea and weight loss. Following liver transplantation, the DLT donor went into remission for 4 years, but eventually progressed and died. The DLT recipient was re-transplanted with a healthy liver, but amyloidosis progressed after 5 years, with symptoms resembling the progression of the ATTRm liver donor. Proteomic analysis was utilized to type the amyloid deposits present in the DLT recipient fat aspirate specimen obtained prior to the second transplantation and stomach biopsy obtained at disease progression. The fat amyloid deposits contained almost equal amounts of mutant TTR and wild-type TTR peptides, but the stomach biopsy showed only wild-type TTR. There is a high concordance of disease phenotype between inherited and acquired ATTRm, which may help in early recognition of acquired ATTRm in DLT recipients. Liver re-transplantation for acquired ATTRm may not interrupt the cycle of amyloid deposition since normal hepatically produced TTR may co-deposit with trace amount of pre-existing TTRm amyloid.

Original languageEnglish (US)
Pages (from-to)192-197
Number of pages6
JournalJournal of the Neurological Sciences
Volume379
DOIs
StatePublished - Aug 15 2017

Fingerprint

Transplants
Liver
Amyloid Plaques
Amyloidosis
Amyloid
Liver Transplantation
Stomach
Transplantation
Fats
Tissue Donors
Biopsy
Amyloidosis, Hereditary, Transthyretin-Related
Peripheral Nervous System Diseases
Proteomics
Disease Progression
Weight Loss
Diarrhea
Transplant Recipients
Phenotype
Peptides

Keywords

  • Acquired amyloidosis
  • Phenotype
  • Progression
  • Transthyretin

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

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title = "Acquired transthyretin amyloidosis after domino liver transplant: Phenotypic correlation, implication of liver retransplantation",
abstract = "Reports of acquired ATTRm in domino liver transplant (DLT) recipients are infrequent and the approach to management is unknown. We describe two patients, an inherited ATTRm patient who underwent liver transplant and his liver recipient who developed acquired ATTRm 8 years following transplantation. The clinical manifestations of amyloidosis were similar between patients and consisted of peripheral neuropathy, diarrhea and weight loss. Following liver transplantation, the DLT donor went into remission for 4 years, but eventually progressed and died. The DLT recipient was re-transplanted with a healthy liver, but amyloidosis progressed after 5 years, with symptoms resembling the progression of the ATTRm liver donor. Proteomic analysis was utilized to type the amyloid deposits present in the DLT recipient fat aspirate specimen obtained prior to the second transplantation and stomach biopsy obtained at disease progression. The fat amyloid deposits contained almost equal amounts of mutant TTR and wild-type TTR peptides, but the stomach biopsy showed only wild-type TTR. There is a high concordance of disease phenotype between inherited and acquired ATTRm, which may help in early recognition of acquired ATTRm in DLT recipients. Liver re-transplantation for acquired ATTRm may not interrupt the cycle of amyloid deposition since normal hepatically produced TTR may co-deposit with trace amount of pre-existing TTRm amyloid.",
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T1 - Acquired transthyretin amyloidosis after domino liver transplant

T2 - Phenotypic correlation, implication of liver retransplantation

AU - Muchtar, Eli

AU - Grogan, Martha

AU - Dasari, Surendra

AU - Kurtin, Paul J.

AU - Gertz, Morie

PY - 2017/8/15

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N2 - Reports of acquired ATTRm in domino liver transplant (DLT) recipients are infrequent and the approach to management is unknown. We describe two patients, an inherited ATTRm patient who underwent liver transplant and his liver recipient who developed acquired ATTRm 8 years following transplantation. The clinical manifestations of amyloidosis were similar between patients and consisted of peripheral neuropathy, diarrhea and weight loss. Following liver transplantation, the DLT donor went into remission for 4 years, but eventually progressed and died. The DLT recipient was re-transplanted with a healthy liver, but amyloidosis progressed after 5 years, with symptoms resembling the progression of the ATTRm liver donor. Proteomic analysis was utilized to type the amyloid deposits present in the DLT recipient fat aspirate specimen obtained prior to the second transplantation and stomach biopsy obtained at disease progression. The fat amyloid deposits contained almost equal amounts of mutant TTR and wild-type TTR peptides, but the stomach biopsy showed only wild-type TTR. There is a high concordance of disease phenotype between inherited and acquired ATTRm, which may help in early recognition of acquired ATTRm in DLT recipients. Liver re-transplantation for acquired ATTRm may not interrupt the cycle of amyloid deposition since normal hepatically produced TTR may co-deposit with trace amount of pre-existing TTRm amyloid.

AB - Reports of acquired ATTRm in domino liver transplant (DLT) recipients are infrequent and the approach to management is unknown. We describe two patients, an inherited ATTRm patient who underwent liver transplant and his liver recipient who developed acquired ATTRm 8 years following transplantation. The clinical manifestations of amyloidosis were similar between patients and consisted of peripheral neuropathy, diarrhea and weight loss. Following liver transplantation, the DLT donor went into remission for 4 years, but eventually progressed and died. The DLT recipient was re-transplanted with a healthy liver, but amyloidosis progressed after 5 years, with symptoms resembling the progression of the ATTRm liver donor. Proteomic analysis was utilized to type the amyloid deposits present in the DLT recipient fat aspirate specimen obtained prior to the second transplantation and stomach biopsy obtained at disease progression. The fat amyloid deposits contained almost equal amounts of mutant TTR and wild-type TTR peptides, but the stomach biopsy showed only wild-type TTR. There is a high concordance of disease phenotype between inherited and acquired ATTRm, which may help in early recognition of acquired ATTRm in DLT recipients. Liver re-transplantation for acquired ATTRm may not interrupt the cycle of amyloid deposition since normal hepatically produced TTR may co-deposit with trace amount of pre-existing TTRm amyloid.

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