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Remission of light chain proximal tubulopathy in IgG λ-type multiple myeloma by lenalidomide and dexamethasone therapy

  • Shinya KawamotoEmail author
  • Yuji Hidaka
  • Yu Kaneko
  • Hideo Misawa
  • Katsuhiro Nagahori
  • Atsunori Yoshino
  • Takamitsu Okamura
  • Shinichi Ban
  • Yoshihiko Ueda
  • Tetsuro Takeda
Case Report
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Abstract

Light chain proximal tubulopathy is a rare manifestation of monoclonal gammopathy. A 73-year-old Japanese woman was noted to have urinary protein and hypertension on health examination and visited the regional clinic. She was noted to have IgG λ M protein and suspected of multiple myeloma. She was referred to us with massive proteinuria (7.5 g/g creatinine) and Bence Jones proteinuria without renal dysfunction. A renal biopsy revealed no glomerular abnormalities, but a tubular cast was observed partially in tubules without tubular atrophy or a crystalline structure. Direct Fast Scarlet staining was absent both in glomerulus and vascular wall. Immunofluorescence revealed λ light chain (LC) staining in the proximal tubules. Electron microscopy revealed nonspecific findings including increased lysosomes with irregular contours and mottled appearance. A bone marrow biopsy revealed plasma cell proliferation (35%) and multiple myeloma immunoglobulin G λ type. She showed progressive anemia and decrease of eGFR with elevated level of urinary β-2 microglobulin. She was treated with lenalidomide + dexamethasone (Ld). With Ld therapy, she achieved hematologic and nephrologic remission reducing the free LC, λ/κ ratio, urinary protein level, and urinary β-2 microglobulin level.

Keywords

Light chain proximal tubulopathy Multiple myeloma IgG lambda M protein Lenalidomide 

Notes

Acknowledgements

We thank Takuya Okamura, department of pathology for immunostaining and Matsuo Jinnai, Collaborative Research Center for electron microscopy.

Compliance with ethical standards

Conflict of interest

All authors declare no competing interests.

Human and animal rights

This article does not contain any studies with human participants performed by any of the authors.

Informed consent

Informed consent was obtained from participants included in the article.

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Copyright information

© Japanese Society of Nephrology 2019

Authors and Affiliations

  • Shinya Kawamoto
    • 1
    Email author
  • Yuji Hidaka
    • 1
  • Yu Kaneko
    • 1
  • Hideo Misawa
    • 1
  • Katsuhiro Nagahori
    • 1
  • Atsunori Yoshino
    • 1
  • Takamitsu Okamura
    • 2
  • Shinichi Ban
    • 3
  • Yoshihiko Ueda
    • 3
  • Tetsuro Takeda
    • 1
  1. 1.Department of NephrologyDokkyo Medical University Saitama Medical CenterKoshigayaJapan
  2. 2.Department of Internal MedicineDokkyo Medical University Saitama Medical CenterKoshigayaJapan
  3. 3.Department of PathologyDokkyo Medical University Saitama Medical CenterKoshigayaJapan

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