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Case Reports
. 2024 Mar 1;63(5):693-698.
doi: 10.2169/internalmedicine.1835-23. Epub 2023 Jul 12.

Improvement of Light Chain Proximal Tubulopathy without Crystals in IgGλ-type Monoclonal Gammopathy of Undetermined Significance Using Bortezomib and Dexamethasone

Tomohisa Tsuyuki  1 , Tadashi Uramatsu  2 , Masatoshi Shimizu  1 , Takuma Ishi  1 , Kiyokazu Tsuji  2 , Jun Nakashima  3 , Eisuke Katafuchi  4 , Toshiyuki Nakayama  4 , Noriko Uesugi  5 , Kumiko Muta  1 , Tomoya Nishino  1
Affiliations
Case Reports

Improvement of Light Chain Proximal Tubulopathy without Crystals in IgGλ-type Monoclonal Gammopathy of Undetermined Significance Using Bortezomib and Dexamethasone

Tomohisa Tsuyuki et al. Intern Med. .

Abstract

A 70-year-old woman with acute kidney injury, a high serum Creatinine (Cr) level (3.91 mg/dL), and proteinuria (protein/Cr ratio 1.59 g/gCr) was admitted. Serum IgG λ-type and urinary λ-type M proteins were observed. A bone marrow examination indicated monoclonal gammopathy of undetermined significance (MGUS). A renal biopsy showed distended proximal tubular cells, and immunofluorescence identified tissue positive for proximal tubular cell λ light chains. Electron microscopy identified fibril-like structures in the lysosomes. The patient was diagnosed with light chain proximal tubulopathy without crystals in IgG λ-type MGUS and treated with bortezomib and dexamethasone therapy, which improved her renal function.

Keywords: light chain proximal tubulopathy without crystals; monoclonal gammopathy of renal significance; monoclonal gammopathy of undetermined significance; paraprotein-related kidney disease.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Light microscopy findings. (a) Distended proximal tubular cells and infiltration of inflammatory cells in part of the interstitium (PAS stain; ×40). (b) Proximal tubular cell vacuolation containing granular inclusions (PAS stain; ×400). (c) Hematoxylin and Eosin (H&E) staining highlights of the granular inclusions in the proximal tubular cells (H&E staining; ×400). (d) Acute tubular injury with flattened epithelial cells (PAS stain; ×100). PAS: periodic acid Schiff
Figure 2.
Figure 2.
Immunofluorescence staining for κ and λ light chains on protease-digested proximal tubules. κ light chains were negative, but λ light chains were positive in the proximal tubules.
Figure 3.
Figure 3.
Electron microscopy analysis results. Electron microscopy shows lysosomes, including fibril-like structures, in the proximal tubules (a, b).
Figure 4.
Figure 4.
Clinical course of the patient. Serum Cr levels improved to 1.35 mg/dL, the urinary protein/Cr ratio improved to 0.82 g/gCr, and serum λ light chain levels decreased to 29.2 mg/L after 7 months of treatment.
See this image and copyright information in PMC

References

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