Multiple Myeloma and Related Disorders

Multiple myeloma is a clonal plasma cell malignancy. Major advances have occurred in the last decade in the diagnosis and treatment of myeloma including new diagnostic criteria, revised staging systems, updated response criteria, and the development of several new drugs. Six new drugs-carfilzomib, pomalidomide, panobinostat, elotuzumab, daratumumab, and ixazomib-were approved for the treatment of relapsed and/or refractory myeloma by the US Food and Drug Administration (FDA) in the last few years. As a result, overall survival has improved substantially. Current initial therapy consists of induction with a regimen such as bortezomib, lenalidomide, and dexamethasone (VRd) followed by autologous stem cell transplantation, followed by maintenance therapy with either lenalidomide (standard-risk patients) or bortezomib (high-risk patients). Median survival is approximately 6 to 7 years. An array of related plasma cell disorders need to be distinguished from myeloma, including monoclonal gammopathy of undetermined significance, smoldering multiple myeloma, Waldenström macroglobulinemia, solitary plasmacytoma, and AL amyloidosis.