Kidney injury related to paraproteinemia is common and typically occurs after the 4th decade of life in association with an underlying plasma cell dyscrasia or other lymphoproliferative disease. Kidney transplantation in paraprotein-related kidney disease can be successful in conjunction with treatment of the underlying hematopoietic process; however, when hematologic response to therapy is not achieved, recurrent kidney injury is frequently seen. We describe a young male patient who presented with end stage kidney disease at the age of 23 years thought to be secondary to focal segmental glomerulosclerosis who ultimately received two kidney allografts. He experienced recurrent proteinuria in both kidneys with a biopsy from his second allograft showing kappa-restricted crystalline light chain podocytopathy, which was identified in both his native and first allograft kidneys upon retrospective review. Recurrent light chain podocytopathy has not been previously reported but poses a diagnostic challenge as it can mimic focal segmental glomerulosclerosis, particularly in young patients where paraprotein-related kidney injury is usually not suspected.
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