Kappa light chain nephropathy without evidence of myeloma cells. Response to chemotherapy with cessation of maintenance hemodialysis.
A rarely diagnosed nodular glomerulopathy is presented arising secondary to kappa light chain deposition and clinically characterized by hypertension, congestive heart failure, massive proteinuria and slowly progressive azotemia. Kappa light chains were detected in the urine, the glomerular nodules, and the basement membranes of both glomeruli and tubules. A malignant proliferation of plasma cells could not be detected. Two morphologic features were unusual: the presence of microaneurysms, and the deposition of immunoglobulin and complement in a similar pattern to the kappa light chains. Noteworthy clinical aspects included the elusiveness of the proper diagnosis, the massive proteinuria in the absence of amyloid deposits, and the remarkable improvement in renal function following intermittent chemotherapy.
D000368:Aged; D005260:Female; D006801:Humans; D007105:Immune Complex Diseases / Q000188:drug therapy* / Q000473:pathology; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007676:Kidney Failure, Chronic / Q000188:drug therapy* / Q000473:pathology; D007678:Kidney Glomerulus / Q000473:pathology; D008558:Melphalan / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D006435:Renal Dialysis; D014750:Vincristine / Q000627:therapeutic use
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D014750:Vincristine; D008558:Melphalan; D011241:Prednisone