Manifestations of systemic light chain deposition.
In two patients with terminal renal failure, manifestations of disease developed in multiple organ systems. One had a previous diagnosis of multiple myeloma with kappa light chain proteinemia and proteinuria. The other had idiopathic lobular glomerulonephritis. Hepatic and neurologic abnormalities developed in both; in the latter gastrointestinal, cardiac and endocrine disease developed as well. Clinical and pathologic correlations suggest that the retention and tissue deposition of light chains produced the organ dysfunction, inasmuch as free kappa light chain determinants were demonstrated histologically in the clinically affected organs. The deposition in these patients may be an extreme example of a common but previously unrecognized form of plasma cell dyscrasia.
D000328:Adult; D000368:Aged; D001485:Basement Membrane / Q000276:immunology; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007668:Kidney / Q000276:immunology; D007676:Kidney Failure, Chronic / Q000276:immunology; D007678:Kidney Glomerulus / Q000276:immunology; D008297:Male; D009101:Multiple Myeloma / Q000276:immunology*
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains