LIGHT CHAIN PROXIMAL TUBULOPATHY ASSOCIATED WITH CONCOMITANT FANCONI AND NEPHROTIC SYNDROMES IN A PATIENT WITH MULTIPLE MYELOMA

 

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https://storage.unitedwebnetwork.com/files/1099/e3c1a398112482561be923d2c473eded.pdf
LIGHT CHAIN PROXIMAL TUBULOPATHY ASSOCIATED WITH CONCOMITANT FANCONI AND NEPHROTIC SYNDROMES IN A PATIENT WITH MULTIPLE MYELOMA

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Akiko
Okamoto
Akiko Okamoto iamakiko50@gmail.com Tokai University Division of Nephrology, Endocrinology, and Metabolism Kanagawa Japan *
Yosuke Nakagawa ysuke-nkgw@tokai.ac.jp Tokai University Division of Nephrology, Endocrinology, and Metabolism Kanagawa Japan -
Kazumi Kozuka kozuka.kazumi.h@tokai.ac.jp Tokai University Division of Nephrology, Endocrinology, and Metabolism Kanagawa Japan -
Kenichi Oikawa oikawa.kenichi.m@tokai.ac.jp Tokai University Division of Nephrology, Endocrinology, and Metabolism Kanagawa Japan -
Tomoyuki Toya toya.tomoyuki.m@tokai.ac.jp Tokai University Division of Nephrology, Endocrinology, and Metabolism Kanagawa Japan -
Norisuke Shimamura rfj9563@tokai.ac.jp Tokai University Division of Nephrology, Endocrinology, and Metabolism Kanagawa Japan -
Masahiro Koizumi km9883@tokai.ac.jp Tokai University Division of Nephrology, Endocrinology, and Metabolism Kanagawa Japan -
Go Ogura iamakiko50@gmail.com Tokai University Division of Pathology Kanagawa Japan -
Hirotaka Komaba hkomaba@tokai.ac.jp Tokai University Division of Nephrology, Endocrinology, and Metabolism Kanagawa Japan -
 
 
 
 
 
 

Light chain proximal tubulopathy (LCPT) is a rare form of myeloma-related kidney disease characterized by cytoplasmic inclusions of monoclonal light chains within proximal tubular cells. LCPT frequently presents with Fanconi syndrome, whereas nephrotic-range proteinuria is rarely observed. Here, we report a case of LCPT associated with concomitant Fanconi and nephrotic syndromes in a patient with multiple myeloma.

A 73-year-old woman with systemic sclerosis was found to have progressive proteinuria without an increase in serum creatinine levels (1.1–1.2 mg/dL). Her proteinuria and hypoalbuminemia gradually worsened, eventually leading to the development of nephrotic syndrome. Laboratory findings showed glycosuria, elevated urinary β₂MG and NAG levels, hypokalemia, hypophosphatemia, and hypouricemia, consistent with Fanconi syndrome. She also had anemia with rouleaux formation, elevated serum IgG (2182 mg/dL), and monoclonal IgG with κ light chains on serum immunoelectrophoresis. Bone marrow biopsy showed that 12.6% plasma cells, leading to the diagnosis of multiple myeloma. Kidney biopsy demonstrated swollen proximal tubules with κ light chain-restricted cytoplasmic inclusions. Electron microscopy revealed phagolysosomes containing electron-dense deposits in the cytoplasm of proximal tubular cells, confirming the diagnosis of LCPT. Bortezomib and dexamethasone therapy was initiated, followed by daratumumab-based regimens. Proteinuria gradually decreased from 8.2 to 2.2 g/gCr over two years, while renal function remained stable.


 This case suggests that LCPT can present with nephrotic-range proteinuria, presumably due to a combination of light chain overflow and impaired tubular reabsorption, even in the absence of glomerular involvement.

This abstract includes content previously presented at “The Japanese Society of Internal Medicine, Koto-Hajime 2025 (Osaka, Japan)”. Re-submission to WCN 2026 is permitted by the organizers of the original meeting.

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