Progressive wild-type transthyretin deposition after liver transplantation preferentially occurs onto myocardium in FAP patients.
To elucidate whether progressive wild-type transthyretin (TTR) deposition can actually occur after liver transplantation (LT), amyloid fibrils were investigated in two familial amyloid polyneuropathy patients with TTR Val30Leu variant, who died 1 year after LT. Amyloid fibrils were extracted from cardiac muscles, sciatic nerves and kidney, which were investigated by the immunoprecipitation-mass spectrometry method and liquid chromatography-ion trap mass spectrometry analysis. The ratio of wild-type to variant TTR in cardiac muscle was approximately 5:5 before LT, but greatly increased to about 9:1 after transplantation. The ratios in sciatic nerves and kidney obtained at autopsy were approximately 5:5. Wild-type TTR was undetectable in kidney amyloid obtained before LT. Our results indicate that paradoxical wild-type TTR deposition after LT can preferentially occur in myocardium, leading to fatal cardiac dysfunction, but it is quite likely that this phenomenon can also occur in other visceral organs.
Yazaki
M
M
Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan. mayazaki@hsp.md.shinshu-u.ac.jp
Mitsuhashi
S
S
Tokuda
T
T
Kametani
F
F
Takei
Y-I
YI
Koyama
J
J
Kawamorita
A
A
Kanno
H
H
Ikeda
S-I
SI
D028227:Amyloid Neuropathies, Familial; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism*; D011228:Prealbumin / Q000378:metabolism*
D011228:Prealbumin