Orthotopic liver transplantation, by eliminating the major site of amyloido
genic protein synthesis, is currently the only definitive treatment of most
hereditary amyloidoses. Because of the minimal parenchymal involvement, th
e explanted livers from familial amyloidotic polyneuropathy (FAP) patients
have been transplanted into non-FAP patients in a "domino" fashion. The aim
of this study was to evaluate the extent of amyloid deposits in explanted
livers from two patients with apolipoprotein A-I amyloidosis, with the Arg2
6 mutation, to determine their suitability as domino donors. A detailed his
tologic review of the explanted livers from two patients was performed and
assessed for the extent of amyloid deposition by routine and Congo red stai
ns. Both patients had identical histopathologic features. The liver parench
ymal involvement was strikingly severe. Large patches of amyloid separated
hepatic cords, with accentuation around the central veins. Ah portal triads
were consistently and markedly involved with amorphous eosinophilic deposi
ts within the connective tissue compressing the bile ducts and vascular str
uctures. Hilar vessels had patchy deposits. No involvement of hilar nerve b
ranches was seen. The hepatic parenchyma is extensively involved in heredit
ary Apolipoprotein A-I amyloidosis, with the Arg26 mutation. These livers,
removed at orthotopic liver transplantation, are not suitable for domino do
nation.