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

Area of Science:

• Nephrology
• Immunology
• Pathology

Background:

• Light chain deposition disease (LCDD) is a rare complication of monoclonal gammopathy, often leading to renal failure.
• Recurrent LCDD post-renal transplantation presents unique challenges in long-term patient management.
• Previous reports have not detailed the long-term clinical course and renal pathology of recurrent LCDD after kidney transplantation.

Observation:

• This report details the long-term clinical course and renal pathology of a single patient with recurrent immunoglobulin A λ-type LCDD post-renal transplantation.
• The patient achieved initial remission after bortezomib and dexamethasone therapy, with biopsy findings showing residual nodular lesions but resolution of subendothelial expansion.
• After 6 years of remission, the patient experienced a late relapse with increased proteinuria and decreased renal function, evidenced by advanced nodular formation and subendothelial expansion on biopsy.

Findings:

• Recurrent LCDD can manifest with late relapses, even after achieving complete serological remission.
• Renal pathology in recurrent LCDD can progress over time, with advanced nodular formation and subendothelial expansion observed in later biopsies.
• Long-term remission does not preclude the possibility of late recurrence in LCDD patients post-renal transplantation.

Implications:

• Protocol biopsy monitoring may be necessary for kidney transplant recipients with a history of LCDD to detect late relapses.
• Understanding the long-term pathological changes in recurrent LCDD is crucial for refining treatment strategies.
• This case underscores the importance of vigilant, long-term follow-up for patients with monoclonal gammopathies and kidney transplants.