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Kidney Week

Abstract: FR-PO1082

Multiple Deposition Phenotypes of Monoclonal Immunoglobulin Deposition Disease on Renal Biopsy: Significance of Treatment

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Adapa, Sreedhar R., The Nephrology Group, Inc, Fresno, California, United States
  • Nast, Cynthia C., Cedars-Sinai Medical Center, Los Angeles, California, United States
  • Dhingra, Hemant, The Nephrology Group, Inc, Fresno, California, United States
Introduction

Plasma cell dyscrasias frequently involve the kidney causing renal dysfunction. Various morphologies occur in kidneys, infrequently with several manifestations in one biopsy. We present a case, which revealed multiple pathological features on same biopsy, which responded to treatment.

Case Description

A 61 year old female presented with fatigue and dyspnea for 3 months. She had new onset renal failure with creatinine 3.0 mg/dL and proteinuria. Serological work up revealed monoclonal gammopathy with elevated K:L ratio of 1394. Renal biopsy revealed several paraprotein manifestations including kappa light chain deposition disease, monoclonal fibrillary glomerulonephritis, cryocrystalglobulenemia and fibrillary/tubular cast nephropathy. There was also incidental LECT 2 amyloidosis, negative for kappa light chain and confirmed by mass spectrometry. Bone marrow biopsy revealed 10-20% kappa restricted plasma cells. She received 8 cycles of CyBorD (Cyclophosphamide, Bortizomib and Dexamethasone) chemotherapy. Renal function improved with the recent creatinine 1.1 mg/dL and decreased proteinuria. K:L ratio improved to 1.7. Repeat bone marrow biopsy showed no evidence of abnormal plasma cells by immunohistochemistry. The patient is currently being evaluated for an autologous stem cell transplant.

Discussion

The finding of several concurrent morphologic manifestations of light chain deposition in one biopsy suggests local microenvironment effects in addition to structural properties of the light chain. The renal recovery demonstrates responsiveness to chemotherapy irrespective of the form of light chain deposition noted on biopsy. In this setting, amyloid may not always be light chain related, and other amyloid types should be considered when light chain staining is discrepant.