Multiple myeloma: 2012 update on diagnosis, risk‐stratification, and management

Disease overview: Multiple myeloma accounts for ∼10% of all hematologic malignancies. Diagnosis: The diagnosis requires 10% or more clonal plasma cells on bone marrow examination or a biopsy proven plasmacytoma plus evidence of end‐organ damage felt to be related to the underlying plasma‐cell disorder. Risk stratification: Patients with 17p deletion, t(14;16), t(14;20), or high‐risk gene expression profiling signature have high‐risk myeloma. Patients with t(4;14) translocation, karyotypic deletion 13, or hypodiploidy are considered to have intermediate‐risk disease. All others are considered to have standard‐risk myeloma. Risk‐adapted therapy: Standard‐risk patients are treated with nonalkylator‐based therapy such as lenalidomide plus low‐dose dexamethasone (Rd) followed by autologous stem‐cell transplantation (ASCT). An alternative strategy is to continue initial therapy after stem‐cell collection, reserving ASCT for first relapse. Intermediate‐risk and high‐risk patients are treated with a bortezomib‐based induction followed by ASCT and then bortezomib‐based maintenance. Patients not eligible for ASCT can be treated with Rd for standard risk disease, or with a bortezomib‐based regimen if intermediate‐risk or high‐risk features are present. To reduce toxicity, when using bortezomib, the once‐weekly subcutaneous dose is preferred; similarly, when using dexamethasone, the low‐dose approach (40 mg once a week) is preferred, unless there is a need for rapid disease control. Management of refractory disease: 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. The most promising new agents in development are pomalidomide and carfilizomib. Am. J. Hematol., 2012. © 2011 Wiley Perodicals, Inc.