Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma

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 pr...

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Detalles Bibliográficos
Autores principales: Kishi, Seiji, Obata, Fumiaki, Miki, Hirokazu, Matsuura, Motokazu, Nishimura, Kenji, Tamaki, Masanori, Kishi, Fumi, Murakami, Taichi, Abe, Hideharu, Nagai, Kojiro, Abe, Masahiro, Doi, Toshio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6355399/
https://www.ncbi.nlm.nih.gov/pubmed/30101939
http://dx.doi.org/10.2169/internalmedicine.1323-18
Descripción
Sumario: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.

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