Recipient aging accelerates acquired transthyretin amyloidosis after domino liver transplantation

Liver Transplantation - Tập 22 Số 5 - Trang 656-664 - 2016
Yohei Misumi1, Yuki Narita2, Toshinori Oshima1, Mitsuharu Ueda1, Taro Yamashita1, Masayoshi Tasaki3,4, Konen Obayashi3, Kaori Isono2, Yukihiro Inomata2, Yukio Ando1
1Departments of Neurology,Kumamoto University,Kumamoto,Japan
2Departments of Transplantation and Pediatric Surgery, Graduate School of Medical Sciences,Kumamoto University,Kumamoto,Japan
3Departments of Morphological and Physiological Sciences, Graduate School of Health Sciences,Kumamoto University,Kumamoto,Japan
4Departments of Morphological and Physiological Sciences, Graduate School of Health SciencesKumamoto UniversityKumamotoJapan

Tóm tắt

Domino liver transplantation (DLT) with liver grafts from patients with hereditary transthyretin (TTR) amyloidosis has been performed throughout the world because of a severe liver graft shortage. Reports of acquired systemic TTR amyloidosis in domino liver recipients have been increasing; however, the precise pathogenesis and clinical course of acquired TTR amyloidosis remains unclear. We analyzed the relationship between the occurrence of acquired amyloidosis and clinical features in 22 consecutive domino liver donors with hereditary TTR amyloidosis (10 males and 12 females; mean age at DLT: 37.2 years; TTR mutations: V30M [n = 19], Y114C [n = 1], L55P [n = 1], and S50I [n = 1]) and 22 liver recipients (16 males and 6 females; mean age at DLT, 46.2 years). The mean times from DLT to amyloid first appearance and transplant recipient symptom onset were 8.2 years and 9.9 years, respectively. Kaplan‐Meier analysis and quantification of the amyloid deposition revealed aging of recipients correlated with early de novo amyloid deposition. The sex of donors and recipients and the age, disease duration, and disease severity of donors had no significant effect on the latency of de novo amyloid deposition. In conclusion, our results demonstrate that recipient aging is associated with the early onset de novo amyloidosis. Because acquired amyloidosis will likely increase, careful follow‐up for early amyloidosis detection and new treatments, including TTR stabilizers and gene‐silencing therapies, are required. Liver Transplantation 22 656‐664 2016 AASLD.

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Liver Transplantation

Tập 22 Số 5

656-664

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