Korean J Nephrol.  1997 Dec;16(4):783-787.

A Case of Light Chain Deposition Disease Associated with Renal Amyoidosis

• Kim G
• Chang KY
• Baek SH
• Kim KD
• Bae SJ
• Kim JS
• Kim SE
• Kim MH
• Kim HJ
• Kim KH

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.

Keyword

Light chain deposition disease; Renal amyloidosis; Nephrotic syndrome

MeSH Terms

Amyloidosis
Basement Membrane
Biopsy
Bone Marrow
Creatinine
Dyspnea
Edema
Electrophoresis
Heart Failure
Humans
Hypertension
Immunoelectrophoresis
Immunoglobulin G
Immunoglobulin Light Chains
Kidney
Kidney Failure, Chronic
Male
Melphalan
Middle Aged
Multiple Myeloma
Nephrotic Syndrome
Paraproteinemias
Prednisone
Proteinuria
Renal Insufficiency
Creatinine
Immunoglobulin G
Immunoglobulin Light Chains
Melphalan
Prednisone