ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: TH-PO0840

A Twisted Tale of Monoclonal Gammopathy of Renal Significance

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Liu, Andy Tianyu, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Ashoka, Ankita, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Patel, Krunal, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Moreno, Vanessa, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
  • Jain, Koyal, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
Introduction

Monoclonal Gammopathy of Renal Significance (MGRS) can vary in kidney manifestations. We report a case of worsening kidney disease in a patient who initially responded well to daratumumab, bortezomib, cyclophosphamide, dexamethasone (DaraCyBorD) for biopsy proven light chain deposition disease (LCDD).

Case Description

52-year-old female with hypertension, smoking, chronic kidney disease 3b diagnosed with LCDD seven months prior to presentation. Initial labs: IgG lambda on immunofixation (IFE), 0.7 g/dL M-spike, serum free light chain (SFLC) kappa 8.11 mg/dL, lambda 7.91 mg/dL and ratio 1.03, normocellular bone marrow. DaraCyBorD treatment was started and, after 3 cycles, had a good hematologic response 0.16 g/dL M-spike, kappa 3.59 mg/dL, lambda 2.01 mg/dL and ratio 1.79. Renal function and proteinuria worsened prompting further evaluation. At presentation, labs showed Cr 4.8 mg/dL (from baseline of ~2.4 mg/dl), UPCR 6.2g/g, IgG Kappa on IFE (likely from Daratumumab), 0.1 g/dL M-spike, kappa 3.64mg/dL, lambda 2.57 mg/dL and ratio 1.04. Repeat kidney biopsy (Figure) showed two concurrent clonal processes causing MGRS: 1) proliferative glomerulonephritis with monotypic immunoglobulin deposition with IgG3 kappa deposits characterized by diffuse proliferative glomerular injury with 20-25% cellular crescents and focal necrotizing lesions, and 2) heavy/light chain deposition disease with IgG1 lambda deposits. She was treated with high-dose steroids, but chemotherapy was deferred pending bone marrow biopsy results.

Discussion

Dual concurrent clonal processes causing MGRS is rare. Typically, non-amyloid MGRS responds to initial treatment in 72% of patients. A second MGRS may represent treatment failure or a previously undetected clone. Clinicians should have a low threshold for repeat kidney biopsy in patients with worsening renal function and proteinuria despite good response to chemotherapy.

Digital Object Identifier (DOI)