An autopsy case of amyloid tubulopathy exhibiting characteristic spheroid-type deposition
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.
KeywordsAmyloidosis Amyloid tubulopathy Kidney Light chain proximal tubulopathy Spheroid structure
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.
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.
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.
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