Light Chain Deposition Disease Recurrence in Renal Allograft after Long-Term Remission

Nephron. 2023:147 Suppl 1:96-100. doi: 10.1159/000529776. Epub 2023 Feb 21.

Abstract

Light chain deposition disease (LCDD) is a rare manifestation of monoclonal gammopathy, which can lead to renal failure. We previously reported a detailed recurrence process in a case of LCDD after renal transplantation. To the best of our knowledge, no report has described the long-term clinical course and renal pathology findings of recurrent LCDD in patients after renal transplantation. In this case report, we describe the long-term clinical presentation and changes in renal pathology of the same patient after early LCDD relapse in a renal allograft. A 54-year-old woman with recurrent immunoglobulin A λ-type LCDD in an allograft was admitted 1 year post-transplant for bortezomib and dexamethasone therapy. At 2 years post-transplantation, a graft biopsy performed after complete remission was achieved, showing some glomeruli with residual nodular lesions similar to the pre-treatment renal biopsy findings. However, the enlarged subendothelial space disappeared. She remained in complete remission serologically for 6 years. Subsequently, the ratio of serum κ/λ-free light chains decreased gradually. She underwent a transplant biopsy approximately 12 years after renal transplantation due to increased proteinuria and decreased renal function. Compared with the previous graft biopsy, almost all glomeruli showed advanced nodule formation and subendothelial expansion. Because the LCDD case relapsed after long-term remission following renal transplantation, protocol biopsy monitoring might be necessary.

Keywords: Allograft kidney; Light chain deposition disease; Recurrent renal disease.

Publication types

  • Case Reports

MeSH terms

  • Allografts / pathology
  • Female
  • Humans
  • Immunoglobulin Light Chains
  • Kidney / pathology
  • Kidney / physiology
  • Kidney Transplantation*
  • Middle Aged
  • Multiple Myeloma* / pathology
  • Multiple Myeloma* / therapy
  • Paraproteinemias* / pathology

Substances

  • Immunoglobulin Light Chains