title: Light Chain Podocytopathy Mimicking Recurrent Focal Segmental Glomerulosclerosis.
abstract: Kidney injury related to paraproteinemia is common and typically occurs after the fourth 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 at the age of 23 years with end-stage kidney disease thought to be secondary to focal segmental glomerulosclerosis; this patient 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 in whom paraprotein-related kidney injury is usually not suspected.
authors: Department of Pathology, University of Utah, Salt Lake City, UT.
authors: M A
authors: MA
authors: Khalighi
authors: Department of Pathology, University of Utah, Salt Lake City, UT.
authors: M P
authors: MP
authors: Revelo
authors: Division of Nephrology, University of Utah, Salt Lake City, UT.
authors: J D
authors: JD
authors: Abraham
authors: Division of Nephrology, University of Utah, Salt Lake City, UT.
authors: F
authors: F
authors: Shihab
authors: Division of Nephrology, University of Utah, Salt Lake City, UT.
authors: F
authors: 0000-0002-1018-0944
authors: F
authors: Ahmed
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D006084:Graft Rejection / Q000175:diagnosis* / Q000209:etiology; D006085:Graft Survival; D006801:Humans; D016030:Kidney Transplantation / Q000009:adverse effects*; D009404:Nephrotic Syndrome / Q000175:diagnosis* / Q000209:etiology; D050199:Podocytes / Q000473:pathology*; D011379:Prognosis; D011507:Proteinuria / Q000175:diagnosis* / Q000209:etiology; D012008:Recurrence; D012189:Retrospective Studies; D055815:Young Adult
keywords: biopsy; cancer/malignancy/neoplasia: hematogenous/leukemia/lymphoma; clinical research/practice; kidney (allograft) function/dysfunction; kidney transplantation/nephrology; pathology/histopathology