A Case of Light Chain Deposition Disease Associated with Renal Amyoidosis / 대한신장학회잡지

ABSTRACT

Light chain deposition disease of kidney is characterized by deposition of monoclonal immunoglobulin light chain and electron-dense material in glomerular and tubular basement membrane and usually associated with multiple myeloma or other plasma cell dyscrasia. With light chain deposition disease affecting kidney, three clinical patterns have been recognized; nephrotic syndrome, rapidly progressive renal failure and slowly progressing chronic renal failure. The majority of patients present proteinuria and renal insufficiency. Cytotoxic therapy has been considered as treatment of choice. Favorable effect of melphalan given together with prednisone has been reported in a few cases. A 64-year-old male was admitted with generalized edema and exertional dyspnea, and was presumptively diagnosed as congestive heart failure and hypertension. He also presented increased serum creatinine and nephrotic range proteinuria. Urine protein electrophoresis and urine and serum immunoelectrophoresis revealed monoclonal gammopathy of IgG kappa type. Work up for multiple myeloma including bone marrow biopsy showed results compatible with smoldering myeloma. Renal biopsy showed findings of light chain deposition disease and Congo-red positive amyloidosis. After we treated the patient with melphalan and predinsone for two cycles, amount of proteinuria and serum creatinine were decreased.