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Abstract: SA-PO857

Relapsing Proliferative Glomerulonephritis With Monoclonal Immunoglobulin Deposits After Renal Transplantation Presenting With AKI and Gross Hematuria

Session Information

Category: Transplantation

  • 2002 Transplantation: Clinical

Authors

  • Shaikh, Sana J., University of California San Francisco, San Francisco, California, United States
  • Laszik, Zoltan G., University of California San Francisco, San Francisco, California, United States
  • Wong, Sandy W., University of California San Francisco, San Francisco, California, United States
  • Webber, Allison B., University of California San Francisco, San Francisco, California, United States
Introduction

Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits (PGNMID) is a subtype of monoclonal gammopathy of renal significance (MGRS), resulting from deposition of intact monoclonal IgG in glomeruli.

Case Description

This is a 62-year-old woman with ESRD attributed to HTN who underwent a living-related renal transplant in 2009. She developed MGRS involving her allograft, diagnosed 7 years post-transplant during workup of AKI and nephrotic range proteinuria. Transplant biopsy showed membranoproliferative glomerulonephritis (MPGN) with gamma-3 heavy chain and kappa light chain restriction. Paraproteinemia workup was normal (SPEP, UPEP, serum and urine IFE, bone marrow aspirate and biopsy, PET-CT), aside from a marginal free light chain ratio (2.5). C3, C4, cryoglobulin, HIV, HBV, HCV were normal/negative. She received induction therapy with cyclophosphamide, bortezomib, dexamethasone (CyBorD), then carfilzomib, lenalidomide, dexamethasone. She transitioned to maintenance therapy with daratumumab and remained on tacrolimus monotherapy. Her renal function was stable on this regimen (Cr 1.7 mg/dL, UPCR 0.2) for the next 6 years.

She then developed AKI and gross hematuria. Imaging and urologic workup were negative. Transplant biopsy was performed and revealed active MPGN. This was further classified as IgG3-kappa PGNMID, a more recently described entity. For her declining renal function, she required hemodialysis. As prior, there was no detectable paraprotein. She was treated with CyBorD. 3 months later dialysis was discontinued. The inciting event leading to her relapse was not clear.

Discussion

PGNMID is a plasma cell disorder associated with a paraprotein causing kidney injury that does not meet criteria for multiple myeloma or lymphoma. The rate of detection of the nephrotoxic monoclonal immunoglobulin in the serum or urine, or of an abnormal bone marrow B cell clone is only 30%. After transplantation, PGNMID has a high recurrence rate and is complicated by frequent relapses and decreased graft survival. Management of this disease requires close collaboration with Hematology and involves targeting the presumed plasma or B cell clone.