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e-Article

Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma
Document Type
Journal Article
Source
Internal Medicine. 2018, 57(24):3597
Subject
IgD λ myeloma
Light Chain Proximal Tubulopathy (LCPT)
paraprotein related kidney disease
Language
English
ISSN
0918-2918
1349-7235
Abstract
Light Chain Proximal Tubulopathy (LCPT) is a rare form of paraprotein-related kidney disease in which monoclonal free light chains damage the proximal renal tubular epithelial cells. We herein report the case of a 78-year-old woman who presented with anemia and kidney dysfunction. Serum and urine protein electrophoresis analyses revealed a monoclonal IgD and λ free light chains. Proximal tubular injury and the accumulation of λ light chains were found by kidney biopsy. Electron microscopy revealed no organized structure suggestive of crystals. LCPT was caused by IgD lambda myeloma and bortezomib and dexamethasone therapy led to very good partial response (VGPR) without a worsening of the kidney function.