Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma
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- Kishi Seiji
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Obata Fumiaki
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Miki Hirokazu
- Division of Transfusion Medicine and Cell Therapy, Tokushima University Hospital, Japan
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- Matsuura Motokazu
- Department of Nephrology, Tokushima Red Cross Hospital, Komatsushima, Japan
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- Nishimura Kenji
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Tamaki Masanori
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Kishi Fumi
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Murakami Taichi
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Abe Hideharu
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Nagai Kojiro
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Abe Masahiro
- Department of Hematology, Endocrinology and Metabolism, Graduate School of Biomedical Sciences, Tokushima University, Japan
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- Doi Toshio
- Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan
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説明
<p>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. </p>
収録刊行物
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- Internal Medicine
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Internal Medicine 57 (24), 3597-3602, 2018-12-15
一般社団法人 日本内科学会
