Organ transplantation in hereditary apolipoprotein AI amyloidosis.
Patients with hereditary apolipoprotein AI (apoAI) amyloidosis often have extensive visceral amyloid deposits, and many develop end-stage renal failure as young adults. Solid organ transplantation to replace failing organ function in systemic amyloidosis is controversial due to the multisystem and progressive nature of the disease and the risk of recurrence of amyloid in the graft. We report the outcome of solid organ transplantation, including dual transplants in 4 cases, among 10 patients with apoAI amyloidosis who were followed for a median (range) of 16 (4-28) and 9 (0.2-27) years from diagnosis of amyloidosis and transplantation, respectively. Eight of 10 patients were alive, seven with a functioning graft at censor. Two patients died, one of disseminated cytomegalovirus infection 2 months after renal transplantation and the other of multisystem failure following severe trauma more than 13 years after renal transplantation. The renal transplant of one patient failed due to recurrence of amyloid after 25 years. Amyloid disease progression was very slow and the natural history of the condition was favorably altered in both cases in which the liver was transplanted. Failing organs in hereditary apoAI amyloidosis should be replaced since graft survival is excellent and confers substantial survival benefit.
Gillmore
J D
JD
National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Hampstead Campus, Royal Free and University College Medical School, London, UK. j.gillmore@medsch.ucl.ac.uk
Stangou
A J
AJ
Lachmann
H J
HJ
Goodman
H J
HJ
Wechalekar
A D
AD
Acheson
J
J
Tennent
G A
GA
Bybee
A
A
Gilbertson
J
J
Rowczenio
D
D
O'Grady
J
J
Heaton
N D
ND
Pepys
M B
MB
Hawkins
P N
PN
D000293:Adolescent; D000328:Adult; D028226:Amyloidosis, Familial / Q000097:blood / Q000150:complications* / Q000601:surgery; D016632:Apolipoprotein A-I / Q000097:blood / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006801:Humans; D007676:Kidney Failure, Chronic / Q000097:blood / Q000209:etiology / Q000601:surgery*; D016030:Kidney Transplantation; D017093:Liver Failure / Q000097:blood / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009154:Mutation; D012189:Retrospective Studies; D012307:Risk Factors; D055502:Secondary Prevention; D013997:Time Factors; D016896:Treatment Outcome
D016632:Apolipoprotein A-I