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Regression of renal amyloid deposits by VAD therapy plus autologous stem cell transplantation in a patient with primary AL amyloidosis

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Abstract

We report a 58-year-old Japanese woman who presented with nephrotic syndrome. Steroid therapy and cyclosporine A administration were initiated, but hematological remission and renal response were not achieved. Renal biopsy revealed amyloid deposits in the mesangial region and the small arteries. Proteomic analysis based on laser microdissection and mass spectrometry showed that the amyloid deposits were composed of the constant region of the lambda light chain. She received vincristine, adriamycin, and dexamethasone therapy followed by high-dose melphalan and autologous stem cell transplantation, resulting in hematological complete remission and renal response with negative urinary Bence-Jones protein and proteinuria. Renal biopsy was performed four times during follow-up, demonstrating that amyloid deposits decreased gradually, while glomeruli showing global sclerosis increased from 3 to 62%. This case suggests that glomerular amyloid deposits can be cleared via tissue remodeling, if stem cells producing amyloid precursors are completely replaced by unrelated cells after stem cell transplantation.

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Abbreviations

AL:

Amyloid light chain

VAD:

Vincristine, adriamycin, and dexamethasone

HDM:

High-dose melphalan

ASCT:

Autologous stem cell transplantation

PSL:

Prednisolone

CyA:

Cyclosporine A

BJP:

Bence-Jones protein

PAM:

Periodic acid methenamine silver

PAS:

Periodic acid-Schiff

PAM:

Periodic acid silver methenamine

LMD/MS:

Laser microdissection and mass spectrometry

RBx:

Renal biopsy

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Acknowledgements

We are grateful to Masanori Suzuki for performing electron microscopy.

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Authors and Affiliations

  1. Nephrology Center, Toranomon Hospital Kajigaya, 1-3-1, Takatsu, Kawasaki, 212-0015, Kanagawa, Japan

    Naoya Toriu, Naoki Sawa, Rikako Hiramatsu, Hiroki Mizuno, Daisuke Ikuma, Akinari Sekine, Noriko Hayami, Keiichi Sumida, Masayuki Yamanouchi, Eiko Hasegawa, Junichi Hoshino, Kenmei Takaichi & Yoshifumi Ubara

  2. Department of Hematology, Toranomon Hospital, Tokyo, Japan

    Atsushi Wake & Takeshi Fujii

  3. Department of Pathology, Toranomon Hospital, Tokyo, Japan

    Kenichi Ohashi

  4. Okinaka Memorial Institute for Medical Research, Tokyo, Japan

    Kenmei Takaichi & Yoshifumi Ubara

  5. Department of Pathology, Yokohama City Hospital, Yokohama, Kanagawa, Japan

    Kenichi Ohashi

Authors
  1. Naoya Toriu
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  2. Naoki Sawa
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  13. Atsushi Wake
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  14. Kenichi Ohashi
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  15. Takeshi Fujii
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  16. Yoshifumi Ubara
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Corresponding author

Correspondence to Naoya Toriu.

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The authors also declare that they have no conflicts of interest.

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The present study adhered to the Declaration of Helsinki and the patient gave written consent for the details of her case to be published.

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Toriu, N., Sawa, N., Hiramatsu, R. et al. Regression of renal amyloid deposits by VAD therapy plus autologous stem cell transplantation in a patient with primary AL amyloidosis. CEN Case Rep 9, 6–10 (2020). https://doi.org/10.1007/s13730-019-00416-1

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  • DOI: https://doi.org/10.1007/s13730-019-00416-1

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