title: Living donor kidney and autologous stem cell transplantation for primary systemic amyloidosis (AL) with predominant renal involvement.
abstract: Primary systemic amyloidosis (AL) is characterized by multiorgan deposition of monoclonal immunoglobulin light chain. Renal involvement is common and impaired kidney function is associated with reduced median survival. Autologous stem cell transplantation (SCT) for AL achieves superior response rates compared to chemotherapy alone but patients with end-stage renal disease (ESRD) may be excluded from consideration. A treatment approach consisting of living donor kidney transplantation (LDKTx) followed by autologous SCT was developed for AL with ESRD. Eight patients underwent LDKTx with immediate graft function. Two suffered unanticipated complications post-KTx and died 10 and 3 months later. Two cases of subclinical acute cellular rejection (ACR) and one case of clinical ACR occurred--all reversible with corticosteroid. Six patients had successful stem cell harvests performed and five of these underwent SCT with satisfactory trilineage engraftment. Renal function remained stable following SCT in four and was reduced in one due to infectious and bleeding complications. One patient, who has thus far elected not to undergo SCT, has proteinuria and histologic evidence of recurrent renal amyloidosis. This experience supports the feasibility of sequential living donor KTx and autologous SCT for carefully selected patients with ESRD due to AL.
authors.affiliation: Department of Internal Medicine, Division of Nephrology and Hypertension, Mayo Clinic and Foundation, Rochester, MN, USA. leung.nelson@mayo.edu
authors.forename: Nelson
authors.initials: N
authors.lastname: Leung
authors.forename: Matthew D
authors.initials: MD
authors.lastname: Griffin
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.forename: Eric N
authors.initials: EN
authors.lastname: Haugen
authors.forename: James M
authors.initials: JM
authors.lastname: Gloor
authors.forename: Thomas R
authors.initials: TR
authors.lastname: Schwab
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Textor
authors.forename: Martha Q
authors.initials: MQ
authors.lastname: Lacy
authors.forename: Mark R
authors.initials: MR
authors.lastname: Litzow
authors.forename: Fernando G
authors.initials: FG
authors.lastname: Cosio
authors.forename: Timothy S
authors.initials: TS
authors.lastname: Larson
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
authors.forename: Mark D
authors.initials: MD
authors.lastname: Stegall
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000601:surgery*; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D033581:Stem Cell Transplantation; D013234:Stem Cells; D020858:Tissue and Organ Harvesting; D014182:Transplantation, Autologous; D016896:Treatment Outcome