title: Outcomes and treatments of patients with immunoglobulin light chain amyloidosis who progress or relapse postautologous stem cell transplant.
abstract: Immunoglobulin light chain (AL) Amyloidosis is a condition whereby misfolded proteins generated by plasma cells deposit in tissues causing organ dysfunction. Chemotherapy and autologous stem cell transplant when eligible are standard treatment options. Several studies report long-term outcomes of patients post-transplant. However, there is a paucity of literature describing outcomes of relapsed patients post-transplant. We performed a retrospective study to assess outcomes and therapies employed upon relapse after transplant. Between 1996 and 2009, 410 patients received transplant at the Mayo Clinic as first-line therapy. Of those patients, 42 (10%) died within 3 months of transplant, 64 (16%) died without documented relapse, 158 (38%) were alive without documented progression, and 146 (36%) had documented progression. Those 146 patients are the subject of our study, and their median time to hematologic relapse/progression was 23.6 months (95%CI 18.3, 26.3 months). Their median overall survival and 5-yrs overall survival from post-transplant relapse/progression was 51.7 months (95%CI 34.1-62.3) and 39%, respectively. The most common first regimen for treatment after relapse was lenalidomide or thalidomide. In conclusion, our study indicates that patients with AL amyloidosis fare well post-transplant relapse/progression. Additionally, it provides a yardstick to design clinical trials to determine best treatment options.
authors: Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN, USA.
authors: Rahma
authors: R
authors: Warsame
authors: Soo Mee
authors: SM
authors: Bang
authors: Shaji K
authors: SK
authors: Kumar
authors: Morie A
authors: MA
authors: Gertz
authors: Martha Q
authors: MQ
authors: Lacy
authors: Francis
authors: F
authors: Buadi
authors: David
authors: D
authors: Dingli
authors: Suzanne R
authors: SR
authors: Hayman
authors: Prashant
authors: P
authors: Kapoor
authors: Robert A
authors: RA
authors: Kyle
authors: Nelson
authors: N
authors: Leung
authors: John A
authors: JA
authors: Lust
authors: Stephen J
authors: SJ
authors: Russell
authors: Thomas E
authors: TE
authors: Witzig
authors: Steven R
authors: SR
authors: Zeldenrust
authors: S Vincent
authors: SV
authors: Rajkumar
authors: Angela
authors: A
authors: Dispenzieri
mesh_terms: D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000378:metabolism* / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D007147:Immunoglobulin Light Chains
keywords: amyloidosis; prognosis; relapse; transplant; treatment