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 8years 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 4years, but eventually progressed and died. The DLT recipient was re-transplanted with a healthy liver, but
amyloidosis progressed after 5years, 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.