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An autopsy case of amyloid tubulopathy exhibiting characteristic spheroid-type deposition

  • Shojiro Ichimata
  • Yukiko Hata
  • Ryuta Abe
  • Tsuneaki Yoshinaga
  • Nagaaki Katoh
  • Fuyuki Kametani
  • Masahide Yazaki
  • Yoshiki Sekijima
  • Takashi Ehara
  • Naoki NishidaEmail author
Brief Report

Abstract

An 84-year-old woman with a history of haemodialysis for renal failure from approximately 1 year before death. Autopsy revealed numerous spheroid-type amyloid deposits in the kidney that were observed mainly in the interstitium but not the glomeruli and vessels. In addition, intracytoplasmic small globular amyloid deposits in the proximal tubules in addition to amyloid casts were identified. Immunohistochemistry and proteomic analyses indicated these deposits were composed of λ light chains. Amyloid deposition was also found in the lung and heart. λ-type monoclonal protein was detected in her serum and increased numbers of CD138-positive cells with λ-restriction was observed in the bone marrow. The case was diagnosed as amyloid tubulopathy (AT) associated with systemic ALλ amyloidosis related to plasma cell neoplasm. This case indicates that AT is associated with ALλ amyloidosis, which developed systemically with characteristic amyloid deposition forms. These pathological features may be associated with her rapid progressive renal failure.

Keywords

Amyloidosis Amyloid tubulopathy Kidney Light chain proximal tubulopathy Spheroid structure 

Notes

Acknowledgements

The authors thank Ms. Tamae Sasakura, Mr. Noboru Onozuka, Ms. Syuko Matsumori and Mr. Osamu Yamamoto for their technical assistance. We thank Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript.

Authors’ contributions

S. Ichimata designed the study, performed the histological evaluation and drafted the manuscript and figures; Y. Hata reviewed the manuscript; T. Yoshinaga, N. Katoh, F. Kametani, M. Yazaki and Y. Sekijima analysed the amyloid deposits and reviewed the manuscript; T. Ehara performed electron microscopy and reviewed the manuscript; N. Nishida performed the histological evaluation, evaluated the clinical data and critically reviewed the manuscript.

Funding information

This work was supported in part by JSPS KAKENHI Grant Numbers JP18k10119 to Y.H. and JP17k09263 to N.N.

Compliance with ethical standards

This study was performed in accordance with the ethical standards established in the 1964 Declaration of Helsinki and approved by the Ethics Committee of Toyama University.

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2020

Authors and Affiliations

  • Shojiro Ichimata
    • 1
  • Yukiko Hata
    • 1
  • Ryuta Abe
    • 2
  • Tsuneaki Yoshinaga
    • 2
  • Nagaaki Katoh
    • 2
  • Fuyuki Kametani
    • 3
  • Masahide Yazaki
    • 4
  • Yoshiki Sekijima
    • 2
    • 4
  • Takashi Ehara
    • 5
  • Naoki Nishida
    • 1
    Email author
  1. 1.Department of Legal Medicine, Graduate School of Medicine and Pharmaceutical SciencesUniversity of ToyamaToyamaJapan
  2. 2.Department of Neurology and RheumatologyShinshu University School of MedicineMatsumotoJapan
  3. 3.Department of Dementia and Higher Brain FunctionTokyo Metropolitan Institute of Medical ScienceTokyoJapan
  4. 4.Institute for Biomedical SciencesShinshu UniversityMatsumotoJapan
  5. 5.Department of PathologyShinshu University School of MedicineMatsumotoJapan

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