Multiple Myeloma — NEJM

Review Article

Medical Progress

Multiple Myeloma
Antonio Palumbo, M.D., and Kenneth Anderson, M.D.

N Engl J Med 2011; 364:1046-1060March 17, 2011

Article
Multiple myeloma is a neoplastic plasma-cell disorder that is characterized by clonal proliferation of malignant plasma cells in the bone marrow microenvironment, monoclonal protein in the blood or urine, and associated organ dysfunction.1 It accounts for approximately 1% of neoplastic diseases and 13% of hematologic cancers. In Western countries, the annual age-adjusted incidence is 5.6 cases per 100,000 persons.2 The median age at diagnosis is approximately 70 years; 37% of patients are younger than 65 years, 26% are between the ages of 65 and 74 years, and 37% are 75 years of age or older.2,3 In recent years, the introduction of autologous stem-cell transplantation and the availability of agents such as thalidomide, lenalidomide, and bortezomib have changed the management of myeloma and extended overall survival.3-5 In patients presenting at an age under 60 years, 10-year survival is approximately 30%.4

The Biology of Multiple Myeloma

Myeloma arises from an asymptomatic premalignant proliferation of monoclonal plasma cells that are derived from post–germinal-center B cells. Multistep genetic and microenvironmental changes lead to the transformation of these cells into a malignant neoplasm. Myeloma is thought to evolve most commonly from a monoclonal gammopathy of undetermined clinical significance (usually known as MGUS) that progresses to smoldering myeloma and, finally, to symptomatic myeloma

(Figure 1 Multistep Pathogenesis of Multiple Myeloma.).6 Several genetic abnormalities that occur in tumor plasma cells play major roles in the pathogenesis of myeloma.7

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Multiple Myeloma — NEJM