Dysproteinemia and the Kidney

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Dysproteinemia is a clinical state characterized by abnormal, often excessive, synthesis of immunoglobulin (Ig) molecules or subunits. Dysproteinemia results from clonal proliferation of plasma cells or B lymphocytes. The abnormal circulating Ig molecules or subunits (most commonly free light chains) reach the glomerulus via the systemic circulation and are associated with the development of a variety of pathologic lesions within the kidney. Free light chain molecules may pass through the glomerular basement membrane and form casts within distal tubular lumina (myeloma cast nephropathy) or form crystals within the cytoplasm of proximal tubules (light chain Fanconi syndrome). Alternatively, Ig molecules or subunits may form paraprotein tissue deposits and produce an array of pathologic lesions, most commonly amyloidosis and monoclonal Ig deposition disease. The pattern of renal parenchymal disease is determined by the unique properties of the Ig molecule or subunit. Each of the patterns of renal disease is in turn associated with unique, but frequently overlapping, clinical features and outcomes. This review emphasizes the pathologic, clinical, and prognostic differences among the patterns of renal parenchymal disease related to dysproteinemia.

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