Systemic but asymptomatic transthyretin amyloidosis 8 years after domino liver transplantation

Haruki Koike, Tetsuya Kiuchi, Masahiro Iijima, Mitsuharu Ueda, Yukio Ando, Saori Morozumi, Minoru Tomita, Yuichi Kawagashira, Hirohisa Watanabe, Masahisa Katsuno, Yoshie Shimoyama, Yasumasa Okazaki, Hideya Kamei, Gen Sobue

Research output: Contribution to journalArticlepeer-review

27 Citations (Scopus)


As familial amyloid polyneuropathy (FAP) is an adult-onset disease, a long period is expected between domino liver transplantation (DLT) and the occurrence of amyloidosis in recipients of a FAP liver. However, as time passes, and increased numbers of patients have undergone DLT, patients with symptoms suggesting amyloidosis have been reported. The authors describe, for the first time, pathological findings in an autopsy case of a recipient of a FAP liver. A male patient with primary sclerosing cholangitis received a liver graft from a FAP patient with the transthyretin (TTR) Tyr114Cys mutation when he was 30 years old. Although a recurrence of primary sclerosing cholangitis was detected at age 34, he had no symptoms indicating amyloidosis. He died from Burkitt's lymphoma at 38 years of age. TTR immunoreactive amyloid was found in various organs including the heart, lung, gastrointestinal tract, pancreas, spleen, reproductive system and skeletal muscles. In the nervous system, TTR immunoreactive amyloid deposition was obvious in the sympathetic ganglia and the median nerve within the carpal tunnel, while loss of neurons or nerve fibres was not apparent. This case allows for the characterisation of amyloid deposition during the asymptomatic stage of FAP. Widespread amyloid deposition may occur before tissue damage in this disease.

Original languageEnglish
Pages (from-to)1287-1290
Number of pages4
JournalJournal of Neurology, Neurosurgery and Psychiatry
Issue number11
Publication statusPublished - 11-2011
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health


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