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Chapter 13
Plasma cell dyscrasias
The plasma cell dyscrasias include multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS), plasmacytoma, Waldenström macroglobulinemia (WM), and amyloidosis (AL). These are generally diseases of the elderly, with a median age of onset of over 70 years. MGUSs are many times more common than myeloma, estimated to occur in about 3% of individuals over 70 years of age.
MM is a plasma cell malignancy that characteristically involves extensive infiltration of bone marrow (BM), with the formation of plasmacytomas, as clusters of malignant plasma cells inside or outside of the BM milieu. Consequences of this disease are numerous and involve
Plasma cell development
Etiology and pathogenesis
Diagnostic evaluation
Staging/prognostic factors
Molecular pathogenesis of multiple myeloma
Identification of novel therapeutic targets based upon genetic abnormalities Role of the bone marrow microenvironment in multiple myeloma pathogenesis Role of adhesion molecules Role of cytokines in multiple myeloma Interleukin-6 Insulin-like growth factor 1 VEGF Tumor necrosis factor and CD40 ligandStromal cell-derived factor 1 ![]() Wingless-type mouse mammary tumor virus integration-site family, member 3a Fibroblast growth factor TGF-ß Other cytokines Role of angiogenesis in multiple myeloma Death (apoptotic) signaling in multiple myeloma
Treatment
Initial therapy Intensification therapy Maintenance therapy postintensification Treatment of relapsed and refractory multiple myeloma Supportive care
Other plasma cell disorders
Plasmacytoma Amyloidosis POEMS syndrome Lymphoplasmacytic lymphoma (Waldenström macroglobulinemia) | ||||||||||||||||||||||||||||||||||||||||||||||||||