Risk-adapted autologous stem cell transplantation with adjuvant dexamethasone +/- thalidomide for systemic light-chain amyloidosis: results of a phase II trial

Br J Haematol. 2007 Oct;139(2):224-33. doi: 10.1111/j.1365-2141.2007.06783.x.

Abstract

High-dose melphalan (MEL) with autologous stem cell transplant (SCT) is an effective therapy for systemic AL amyloidosis (AL), but treatment-related mortality (TRM) has historically been high. We performed a phase II trial of risk-adapted SCT followed by adjuvant dexamethasone (dex) and thalidomide (thal) in an attempt to reduce TRM and improve response rates. Patients (n = 45) with newly diagnosed AL involving < or =2 organ systems were assigned to MEL 100, 140, or 200 mg/m(2) with SCT, based on age, renal function and cardiac involvement. Patients with persistent clonal plasma cell disease 3 months post-SCT received 9 months of adjuvant thal/dex (or dex if there was a history of deep vein thrombosis or neuropathy). Organ involvement was kidney (67%), heart (24%), liver/GI (22%) and peripheral nervous system (18%), with 31% having two organs involved. TRM was 4.4%. Thirty-one patients began adjuvant therapy, with 16 (52%) completing 9 months of treatment and 13 (42%) achieving an improvement in haematological response. By intention-to-treat, overall haematological response rate was 71% (36% complete response), with 44% having organ responses. With a median follow-up of 31 months, 2-year survival was 84% (95% confidence interval: 73%, 94%). Risk-adapted SCT with adjuvant thal/dex is feasible and results in low TRM and high haematological and organ response rates in AL patients.

Publication types

  • Clinical Trial, Phase II
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Adjuvants, Immunologic / therapeutic use
  • Adjuvants, Pharmaceutic / therapeutic use
  • Adult
  • Aged
  • Amyloidosis / immunology*
  • Amyloidosis / mortality
  • Amyloidosis / therapy*
  • Combined Modality Therapy
  • Dexamethasone / therapeutic use*
  • Drug Administration Schedule
  • Female
  • Follow-Up Studies
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Immunoglobulin Light Chains*
  • Male
  • Melphalan / therapeutic use
  • Middle Aged
  • Myeloablative Agonists / therapeutic use
  • Proportional Hazards Models
  • Remission Induction
  • Risk Assessment
  • Survival Rate
  • Thalidomide / therapeutic use*
  • Transplantation, Autologous

Substances

  • Adjuvants, Immunologic
  • Adjuvants, Pharmaceutic
  • Immunoglobulin Light Chains
  • Myeloablative Agonists
  • Thalidomide
  • Dexamethasone
  • Melphalan