Highlights
- •Presentation and recurrence of acquired TTR amyloidosis resembles donor's phenotype.
- •Liver re-transplantation for acquired TTR amyloidosis does not halt amyloid deposit.
- •Amyloid deposits after liver re-transplantation are composed of wtTTR.
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.
Abbreviations:
ATTRm (mutated transthyretin amyloidosis), DLT (Domino liver transplant), EF (ejection fraction), GI (Gastrointestinal), IVS (Interventricular septum), LV (left ventricle), MS/MS (tandem mass spectrometry), OLT (Orthotopic liver transplantation), TTR (Transthyretin), TTRm (mutated transthyretin), TTRwt (wild-type transthyretin), TUDCA (Tauroursodeoxycholic acid)Keywords
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Article info
Publication history
Published online: June 13, 2017
Accepted:
June 12,
2017
Received in revised form:
June 8,
2017
Received:
April 6,
2017
Identification
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