Recurrent Light Chain Proximal Tubulopathy in a Kidney Allograft

Andrea Angioi, Hatem Amer, Fernando Custodio Fervenza, Sanjeev M Sethi

Research output: Contribution to journalArticlepeer-review

6 Scopus citations

Abstract

We describe a rare case of light chain proximal tubulopathy developing in a kidney transplant 12 months following transplantation. The patient was known to have a monoclonal gammopathy of undetermined significance (MGUS) for more than 15 years. A kidney biopsy done to determine the cause of decline in kidney transplant function showed light chain proximal tubulopathy characterized by numerous eosinophilic and fuchsinophilic granules in proximal tubular epithelial cells, which stained for κ light chains on pronase-based immunofluorescence studies. Electron microscopy confirmed the diagnosis and showed numerous amorphous and geometrically shaped inclusions in proximal tubular epithelial cells. Evaluation of free light chains revealed markedly elevated κ light chains and bone marrow biopsy showed 5% to 10% κ light chain–restricted plasma cells. Retrospective evaluation of the native kidney biopsy performed 15 years earlier also showed numerous fuchsinophilic granules in proximal tubules that stained brightly for κ light chains on pronase-based immunofluorescence studies. The patient was treated with a regimen of bortezomib and dexamethasone with good partial hematologic response and improvement of kidney function. To summarize, we describe a case of recurrent light chain proximal tubulopathy in the transplant, which is an unusual but important cause of decreased kidney function in the setting of a monoclonal gammopathy.

Original languageEnglish (US)
Pages (from-to)483-487
Number of pages5
JournalAmerican Journal of Kidney Diseases
Volume68
Issue number3
DOIs
StatePublished - Sep 1 2016

Keywords

  • Allograft
  • kidney biopsy
  • light chain proximal tubulopathy (LCPT)
  • monoclonal gammopathy
  • monoclonal gammopathy of undetermined significance (MGUS)
  • multiple myeloma
  • recurrent LCPT
  • renal transplantation

ASJC Scopus subject areas

  • Nephrology

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