Multiple myeloma: 2011 update on diagnosis, risk‐stratification, and management

Disease overview: Multiple myeloma is malignant plasma‐cell disorder that accounts for ∼∼10% of all hematologic malignancies. Diagnosis: The diagnosis requires (1) 10% or more clonal plasma cells on bone marrow examination or a biopsy‐proven plasmacytoma plus (2) evidence of end‐organ damage felt to be related to the underlying plasma cell disorder. Risk stratification: Patients with 17p deletion, t(4;14), t(14;16), t(14;20), and karyotypic deletion 13 or hypodiploidy are considered to have high‐risk myeloma. All others are considered to have standard‐risk disease. 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). If patients are tolerating the induction regimen treatment well, an alternative strategy is to continue initial therapy after stem‐cell collection, reserving ASCT for first relapse. 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 a bortezomib‐based regimen if high‐risk features are present. To reduce toxicity, when using bortezomib, the once‐weekly 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 lenalidomide, bortezomib, or alkylators plus low‐dose corticosteroids. 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. 86:57–65, 2011. © 2010 Wiley‐Liss, Inc.