title: Multiple myeloma: 2013 update on diagnosis, risk-stratification, and management.
abstract: BACKGROUND
  Multiple myeloma accounts for approximately 10% of hematologic malignancies.
  
  
  METHODS
  The diagnosis requires 10% or more clonal plasma cells on bone marrow examination or a biopsy proven plasmacytoma plus evidence of associated end-organ damage. In addition, the presence of 60% or more clonal plasma cells in the marrow is also considered as myeloma regardless of the presence or absence of end-organ damage.
  
  
  METHODS
  In the absence of concurrent trisomies, patients with 17p deletion, t(14;16), and t(14;20) are considered to have high-risk myeloma. Patients with t(4;14) translocation are considered intermediate-risk. All others are considered as standard-risk. RISK-ADAPTED INITIAL THERAPY: Standard-risk patients can be treated with lenalidomide plus low-dose dexamethasone (Rd), or a bortezomib-containing triplet such as bortezomib, cyclophosphamide, dexamethasone (VCD). Intermediate-risk and high-risk patients require a bortezomib-based triplet regimen. In eligible patients, initial therapy is given for approximately 4 months followed by autologous stem cell transplantation (ASCT). Standard-risk patients can opt for delayed ASCT if stem cells can be cryopreserved. In patients are not candidates for transplant, initial therapy is given for approximately 12-18 months.
  After initial therapy, lenalidomide maintenance is considered for standard-risk patients who are not in very good partial response or better, while maintenance with a bortezomib-based regimen should be considered in pateints with intermediate or high-risk myeloma.
  
  
  CONCLUSIONS
  Patients with indolent relapse can be treated first with two-drug or three-drug combinations. Patients with more aggressive relapse often require therapy with a combination of multiple active agents.
authors: Division of Hematology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA. rajkumar.vincent@mayo.edu
authors: S Vincent
authors: SV
authors: Rajkumar
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D019468:Disease Management; D004333:Drug Administration Routes; D004334:Drug Administration Schedule; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D000077269:Lenalidomide; D009101:Multiple Myeloma / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000628:therapy*; D010950:Plasma Cells / Q000473:pathology*; D011379:Prognosis; D011719:Pyrazines / Q000008:administration & dosage; D012008:Recurrence; D012306:Risk; D013792:Thalidomide / Q000008:administration & dosage / Q000031:analogs & derivatives; D014178:Translocation, Genetic; D014182:Transplantation, Autologous
chemical_list: D001897:Boronic Acids; D011719:Pyrazines; D013792:Thalidomide; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide; D000077269:Lenalidomide