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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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
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author Kishi, Seiji
Obata, Fumiaki
Miki, Hirokazu
Matsuura, Motokazu
Nishimura, Kenji
Tamaki, Masanori
Kishi, Fumi
Murakami, Taichi
Abe, Hideharu
Nagai, Kojiro
Abe, Masahiro
Doi, Toshio
author_facet Kishi, Seiji
Obata, Fumiaki
Miki, Hirokazu
Matsuura, Motokazu
Nishimura, Kenji
Tamaki, Masanori
Kishi, Fumi
Murakami, Taichi
Abe, Hideharu
Nagai, Kojiro
Abe, Masahiro
Doi, Toshio
author_sort Kishi, Seiji
collection PubMed
description 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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spelling pubmed-63553992019-02-01 Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma Kishi, Seiji Obata, Fumiaki Miki, Hirokazu Matsuura, Motokazu Nishimura, Kenji Tamaki, Masanori Kishi, Fumi Murakami, Taichi Abe, Hideharu Nagai, Kojiro Abe, Masahiro Doi, Toshio Intern Med Case Report 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. The Japanese Society of Internal Medicine 2018-08-10 2018-12-15 /pmc/articles/PMC6355399/ /pubmed/30101939 http://dx.doi.org/10.2169/internalmedicine.1323-18 Text en Copyright © 2018 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Kishi, Seiji
Obata, Fumiaki
Miki, Hirokazu
Matsuura, Motokazu
Nishimura, Kenji
Tamaki, Masanori
Kishi, Fumi
Murakami, Taichi
Abe, Hideharu
Nagai, Kojiro
Abe, Masahiro
Doi, Toshio
Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma
title Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma
title_full Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma
title_fullStr Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma
title_full_unstemmed Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma
title_short Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma
title_sort lambda light chain non-crystalline proximal tubulopathy with igd lambda myeloma
topic Case Report
url 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
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