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Abstract
Objective To elucidate the clinical characteristics of acquired ATTR amyloidosis after domino liver transplantation (DLT) with liver grafts explanted from patients with hereditary variant ATTR (ATTRv) amyloidosis.
Methods We evaluated the presence of amyloid deposits and clinical symptoms in 30 recipients of domino liver transplants (24 men and 6 women) who underwent DLT with liver grafts explanted from patients with ATTRv amyloidosis. We analyzed symptoms and measures of 7 cases of symptomatic acquired ATTR amyloidosis and compared those with 30 patients with ATTRv amyloidosis who were the domino liver donors. We also reviewed the literature on case studies of acquired ATTR amyloidosis.
Results We found amyloid deposition in 13 of our 30 domino liver recipients. A Kaplan-Meier analysis estimated that the median time from DLT to the first detection of amyloid was 8.5 years. In the literature review, the mean time was 7.3 years, with a wide range of 0.5–13 years. Our 7 symptomatic cases and the literature cases with acquired ATTR amyloidosis presented with clinical features that differed from patients with ATTRv amyloidosis who were the domino liver donors. Patients with acquired ATTR amyloidosis showed markedly milder autonomic disturbance, which is one of the main symptoms of ATTRv amyloidosis.
Conclusions Careful monitoring is required for DLT recipients of ATTRv liver grafts because the time from DLT to disease onset has a wide range and the clinical picture of these DLT recipients is distinct from that of liver donors.
Glossary
- ATTRv=
- hereditary variant ATTR;
- CV-RR=
- coefficient of variation of the R-R interval;
- DLT=
- domino liver transplantation;
- FAP=
- familial amyloid polyneuropathy;
- LT=
- liver transplantation;
- MIBG=
- metaiodobenzylguanidine;
- NCS=
- nerve conduction studies;
- TTR=
- transthyretin
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received February 7, 2019.
- Accepted in final form May 16, 2019.
- © 2019 American Academy of Neurology
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