title: De novo AL amyloidosis in the kidney allograft.
abstract: We report four cases of de novo amyloidosis occurring after 16, 18, 28 and 31 years following kidney transplantation. These patients presented with proteinuria and progressive allograft dysfunction. Kidney biopsy showed AL amyloidosis in all compartments of the allograft kidney. Serum immunofixation studies revealed monoclonal lambda light chains in all four cases. Bone marrow examination showed 10% plasma cells in one case, 5-10% in two cases and less than 5% in one case. Two patients died unexpectedly within 3 months and 1 year of the diagnosis of allograft AL amyloidosis. Of the remaining two, one underwent autologous stem cell transplant that resulted in complete hematologic remission. However, the patient relapsed within 2 years and also developed progressive kidney allograft failure. The patient received a second autologous stem cell transplant with complete hematologic response, followed by a second kidney transplant, which showed no evidence of amyloid at 1-year posttransplant. The remaining case was treated with prednisone and bortezomib, which has stabilized kidney function in the short term. In conclusion, this study shows that AL amyloidosis is an uncommon but important cause of late onset proteinuria in the kidney allograft that results in kidney allograft failure.
authors.affiliation: Department of Medicine, Division of Nephrology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Q
authors.initials: Q
authors.lastname: Qian
authors.forename: S H
authors.initials: SH
authors.lastname: Nasr
authors.forename: M E
authors.initials: ME
authors.lastname: Fidler
authors.forename: L D
authors.initials: LD
authors.lastname: Cornell
authors.forename: S
authors.initials: S
authors.lastname: Sethi
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016030:Kidney Transplantation / Q000009:adverse effects*; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D011507:Proteinuria / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D011719:Pyrazines / Q000008:administration & dosage; D012074:Remission Induction; D016896:Treatment Outcome
chemical_list: D001897:Boronic Acids; D011719:Pyrazines; D000069286:Bortezomib; D008558:Melphalan; D011241:Prednisone