Renal lesions in plasma cell dyscrasias: ultrastructural observations.
The renal biopsies from 47 patients with plasma cell dyscrasias were studied by light and electronmicroscopy, and by immunohistochemical methods. This report is primarily concerned with the ultrastructural features of 24 cases of Bence Jones cast nephropathy and of ten cases of light chain deposit disease. In Bence Jones cast nephropathy, crystals derived from light chain proteins were detected in the majority of cases within the casts or in tubular cells and appeared to be related to the "hard" and "fractured" appearance of the casts as well as to the presence of foreign body type giant cells, the latter probably being of monocyte-macrophage origin. In light chain deposit disease, linear deposits of light chain proteins (eight kappa and two lambda) were present in a subendothelial position along the glomerular basement membrane and along the outer aspect of the tubular basement membranes in all cases, quite often in the mesangial matrix, but much less commonly in the interstitium and in the wall of small arteries. The light and electronmicroscopic features of both Bence Jones cast nephropathy and light chain deposit disease can be considered diagnostic for plasma cell dyscrasia. The possible pathogenetic mechanisms of these two different forms of renal involvement are discussed briefly.
D001536:Bence Jones Protein / Q000032:analysis; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000473:pathology; D007147:Immunoglobulin Light Chains / Q000032:analysis; D007668:Kidney / Q000648:ultrastructure*; D007674:Kidney Diseases / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000473:pathology*
D007147:Immunoglobulin Light Chains; D001536:Bence Jones Protein