Kidney tubular disorders due to monoclonal immunoglobulin light chains are common manifestations of B-cell neoplasm. Cast nephropathy (CN) is the most frequent type of these disorders and may present with acute kidney injury (AKI) due to the presence of excess light chains in the distal tubules. Light chain proximal tubulopathy (LCPT) is an uncommon form of renal disease and may present as Fanconi syndrome due to proximal tubular cell damage by intracellular deposition of light chains. The concomitant disorder of both CN and LCPT is rare given the inherent differences in the biochemical properties of the immunoglobulin light chains of each disorder. We report a 64-year-old man who presented with AKI and Fanconi syndrome who was discovered to have both CN and LCPT due to the underlying disorder of monoclonal gammopathy of renal significance and who has responded favourably with conventional chemotherapy. We also review the existing literature on this interesting subject.
- renal system
- haematology (incl blood transfusion)
- acute renal failure
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Contributors RC helped to report the findings and acquired relevant literature for writing and wrote the manuscript. RSdR helped to report the clinical findings, reviewed and approved the final version of the paper. SB helped to report the clinical findings, reviewed and approved the final version of the paper. IB helped to interpret the pathology findings and significantly improved the discussion and writing of the manuscript. BJ orchestrated the reporting, writing and review of the literature for publication. All the authors have agreed to be accountable for the article and accuracy and integrity of the article.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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