Clinical Nephrology, Volume 106 (2026) - November (408 - 414)

Crystalline κ light-chain proximal tubulopathy and Auer rod-like inclusions presenting as Fanconi syndrome: A case report

Yu Wang4, Li Zhang1, 2, Chang-jiu Liang3, Chang Wang1, 2
1 Department of Nephrology, Institute of Nephrology, Second Affiliated Hospital of Hainan Medical University, 2 Hainan Clinical Research Center for Urinary System Disease, 3 Department of Hematology, Second Affiliated Hospital of Hainan Medical University, Haikou, and 4 Department of Endocrinology and Metabolism, Shenzhen University General Hospital, Shenzhen University, Shenzhen, China

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DOI 10.5414/CN112174

Abstract

We report the case of a 53-year-old man who presented with Fanconi syndrome as the initial clinical manifestation. Laboratory findings included hypokalemia, hypophosphatemia, mild metabolic acidosis, and renal glucosuria. Kidney biopsy revealed crystalline light-chain proximal tubulopathy (LCPT) with κ-restricted light-chain deposition in proximal tubular epithelial cells. Bone marrow examination demonstrated multiple myeloma with plasma cells containing Auer rod-like intracytoplasmic inclusions, an extremely rare morphological finding. The coexistence of crystalline κ-restricted LCPT and Auer rod-like inclusions in plasma cells is exceedingly rare and remains poorly characterized. This case underscores the diagnostic value of kidney biopsy in unexplained Fanconi syndrome and suggests that abnormal physicochemical properties of monoclonal κ light chains may contribute to intracellular crystal formation in both renal tubular epithelial cells and plasma cells.

Author Details

Authors

Departments

  • 1 Department of Nephrology, Institute of Nephrology, Second Affiliated Hospital of Hainan Medical University,
  • 2 Hainan Clinical Research Center for Urinary System Disease,
  • 3 Department of Hematology, Second Affiliated Hospital of Hainan Medical University, Haikou, and
  • 4 Department of Endocrinology and Metabolism, Shenzhen University General Hospital, Shenzhen University, Shenzhen, China

Address

Dr. Chang Wang
Department of Nephrology, Institute of Nephrology
2nd Affiliated Hospital of Hainan Medical University
368 Yehai Ave, Longhua District,
Haikou 570311, People’s Republic of China
Email: [email protected]

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Citation

Yu Wang, Li Zhang, Chang-Jiu Liang, Chang Wang. Crystalline κ light-chain proximal tubulopathy and Auer rod-like inclusions presenting as Fanconi syndrome: A case report
. Clin Nephrol. 2026; 106: 408-414. doi: 10.5414/CN112174. Pubmed: https://pubmed.ncbi.nlm.nih.gov/42610552/; PMID: 42610552.

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