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Abstract: PO1916

Light Chain Deposition Disease (LCDD) in the Setting of Smoldering Myeloma (SM)

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Adewuyi, Joel O., University of Florida, Gainesville, Florida, United States
  • Shah, Chintan Vimalkumar, University of Florida, Gainesville, Florida, United States
  • Modi, Dhruv K., University of Florida, Gainesville, Florida, United States
Introduction

Only 50-60% of patients with LCDD meet the criteria for multiple myeloma (MM). SM, a proliferative plasma cell disorder is a precursor for active symptomatic MM. As LCDD is rare, there is limited data for the treatment of LCDD in the setting of SM.

Case Description

A 49-year-old female was found to have proteinuria and microscopic hematuria during a routine workup. Further evaluation showed proteinuria of 4.0 g/day, serum creatinine of 1.3 mg/dL, kappa light chain (KLC) 94.7 mg/dL, lambda light chain (LLC) 1.57 mg/dL, kappa-Lambda ratio (K/L) 60.24, Hemoglobin 12.8 g/d, Calcium 9.26 mg/dl. Kidney biopsy showed nodular mesangial expansion with mild hypercellularity. Moderate tubular atrophy and interstitial fibrosis. Immunofluorescence showed strong kappa staining of mesangium, glomerular, and tubular basement membrane (TBM) with negative lambda staining. Electron microscopy showed the presence of subendothelial, mesangial, and TBM electron dense deposits. Findings were considered to be consistent with kappa associated LCDD. Bone marrow biopsy showed monoclonal plasma cell population in the bone marrow (5% by flow cytometry and 10-15% by CD138 stain) consistent with smoldering myeloma. FISH was abnormal for monosomy of 13 and 11;14 translocation. The skeletal survey was negative for any lytic lesions. She was treated with Bortezomib/Dexamethasone/Cyclophosphamide based regimen weekly for 8 weeks which resulted in a decrease in KLC to 1.45 mg/dl, LLC to 1.0 g/dl, and K/L ratio to 1.45 with negative serum immunofixation. 24-hour urine protein improved to 2.6 g/d. Serum creatinine remained stable at 1.3 mg/dl. Bone marrow biopsy after chemotherapy showed residual plasma cell myeloma involving 5% of the marrow cells. She underwent high dose melphalan followed by Autologous Stem Cell Transplantation (HDM/ASCT). Follow-up labs six years later confirmed successful treatment with serum creatinine improving to 1.02 mg/dl, 24hr-urine protein 484mg/d without microscopic hematuria. SPEP and UPEP remain negative.

Discussion

We report a case of successfully treated LCDD with high dose chemotherapy followed by HDM/ASCT in the setting of smoldering myeloma with six years of follow-up. Patients with LCDD in smoldering myeloma may benefit from high dose chemotherapy along with HDM/ASCT and it should be considered a treatment option.