Figure 1133

Types of renal involvement in dysproteinemias. The uncontrolled proliferation of a B-cell clone leads to overproduction of a monoclonal immunoglobulin (Ig), either an intact molecule or fragments thereof (light or heavy chains). These molecules can deposit in the kidney and other vital organs, depending on the immunoglobulin class, light or heavy chain isotype, and other only partly understood physiochemi-cal properties. The terminology used in these disorders is sometimes confusing and inconsistent. We use the definitions proposed by Gallo and Kumar [73]. All diseases characterized by deposits of monoclonal immunoglobulin-related material are named monoclonal immunoglobulin deposition diseases (MIDD). These deposits can occur in several forms, as outlined in the figure, and are identified by specific stains (such as congo red) and on immuno-fluorescence and electron microscopy. The histologic and clinical manifestations are dependent on the type of deposition. Included in this overview are fibrillary and immunotactoid glomerulonephritis, which in certain cases also show deposits containing monoclonal immunoglobulins. AH— heavy chain amyloidosis; AL—light chain amyloidosis; GN—glomerulonephritis; HCDD—heavy chain deposition disease; LCDD—light chain DD; LHCDD—light and heavy chain DD.

Pathogenesis of renal lesions in dysproteinemias

Deposition either as light chain, amyloid, or cryglobulins

Cortex

Outer medulla

Inner medulla

Pathogenesis of renal lesions in dysproteinemias

Deposition either as light chain, amyloid, or cryglobulins

Cortex

Outer medulla

Inner medulla

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