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Chapter 20
Plasma cell dyscrasias
Plasma cell dyscrasias include monoclonal gammopathy of undermined significance (MGUS), multiple myeloma (MM), plasmacytoma, Waldenström macroglobulinemia (WM), amyloidosis (AL), and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy monoclonal gammopathy, and skin changes). MGUS, smoldering MM, and symptomatic MM represent a spectrum of the same disease. MGUS is characterized by a serum monoclonal protein <30 g/L, <10% plasma cells in the bone marrow, and absence of end-organ damage (Kyle and Rajkumar, 2009). Smoldering (asymptomatic) MM is characterized by having a serum immunoglobulin (Ig) G or IgA monoclonal protein of 30 g/L or higher and/or 10% or more plasma cells in the
Plasma cell development
Etiology and incidence
Molecular pathogenesis
Genetic and epigenetic regulation of MM Epigenetics in MM Role of the BM microenvironment in MM pathogenesis Role of adhesion molecules Role of cytokines in MM Interleukin-6 Insulin-like growth factor-1 Vascular endothelial growth factor Role of angiogenesis in MM Role of chemokines and cell trafficking in MM Osteoclasts in MM Osteoblasts in MM Diagnostic evaluation Staging/prognostic factors Survival Treatment Initial therapy for patients eligible for SCT Thalidomide in newly diagnosed transplantation-eligible patients Bortezomib in newly diagnosed transplantation-eligible patients Lenalidomide in newly diagnosed transplantation-eligible patients Combination of these agents in newly diagnosed transplantation-eligible patients Induction therapy for non–transplantation-eligible patients Thalidomide Bortezomib Lenalidomide Intensification therapy Maintenance therapy Treatment of relapsed and refractory MM Thalidomide Bortezomib Lenalidomide Supportive care
Plasmacytoma
Other plasma cell disorders
Amyloidosis POEMS syndrome Lymphoplasmacytic lymphoma (WM)
Acknowledgment
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