Korean J Nephrol.  2005 Jan;24(1):146-151.

A Case of Idiopathic Light Chain Deposition Disease

• Lee KW
• Lee HK
• Lee YM
• Na KR
• Suh KS
• Shin YT

Abstract

Light chain deposition disease (LCDD) is a systemic disorder characterized by the deposition of monoclonal immunoglobulin light chains (LCs) in various organs. As LCs are overproduced by an abnormal clone of B cells, LCDD is usually described in the course of plasma cell dyscrasias or other lymphoproliferative disorders. However, it can occur in the absence of any detectable hematological disorder even during prolonged follow-up. We experienced a case of 62-year-woman who presented generalized edema, massive proteinuria and renal insufficiency. The histologic findings showed nodular glomerular mesangial expansion and prominent ribbon like immunohistochemical staining for kappa-light chain in the glomerular capillary wall. There was no evidence of multiple myeloma in bone marrow biopsy specimen. Because she refused the intensive chemothrapy, low dose of prednisolone and cyclophosphamide were prescribed for 6 months. Her renal function was maintained relatively well without renal replacement therapy for 7 months. We report a case of idiopathic LCDD not associated with multiple myeloma or other plasma cell dysclasia.

Keyword

Idiopathic light chain deposition disease; Nephrotic syndrome; Renal insufficiency

MeSH Terms

B-Lymphocytes
Biopsy
Bone Marrow
Capillaries
Clone Cells
Cyclophosphamide
Edema
Follow-Up Studies
Immunoglobulin Light Chains
Lymphoproliferative Disorders
Multiple Myeloma
Nephrotic Syndrome
Paraproteinemias
Plasma Cells
Prednisolone
Proteinuria
Renal Insufficiency
Renal Replacement Therapy
Cyclophosphamide
Immunoglobulin Light Chains
Prednisolone