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Abstract: FR-PO210

Monoclonal Immunoglobulin Deposition Disease in a Young Female

Session Information

Category: Onconephrology

  • 1600 Onconephrology

Authors

  • Sharma Divyadarshini, Divya, Sapthagiri Institute of Medical Sciences and Research Centre, Bangalore, Karnataka, India
  • Sharma Priamvada, Gargi, University of North Carolina System, Chapel Hill, North Carolina, United States
  • Zhang, Pingchuan, Mayo Foundation for Medical Education and Research, Rochester, Minnesota, United States
  • Jain, Koyal, University of North Carolina System, Chapel Hill, North Carolina, United States
Introduction

Monoclonal immunoglobulin deposition disease (MIDD) is characterized by deposition of monoclonal immunoglobulin(MIg) along glomerular and tubular basement membranes (GTBM). Prevalence is higher in males with a mean age of 56 years. Light chain deposition disease (LCDD) accounts for 80% of cases with others being heavy chain(HCDD) and rarely light and heavy chain deposition disease(LHCDD). We present a case of rare pathological features of LHCDD presenting at a young age.

Case Description

A 34 year previously healthy female presented with hypertensive urgency, renal insufficiency and nephrotic syndrome. Urine protein creatinine ratio was 11 g/g, Urine microalbumin creatinine ratio 5.5 g/g, Serum protein electrophoresis (SPEP) revealed MIg - IgG lambda type, serum free light chain (sFLC) ratio of 0.03. Renal biopsy showed LHCDD with membranoproliferative injury and focal cellular crescents, immunofluorescence showed deposits along GTBM that were nonfibrillar by electron microscopy (Figure). Imaging did not suggest multiple myeloma. Bone marrow biopsy showed only 10% plasma cell population. She was treated with cyclophosphamide, bortezomib and dexamethasone (CyBorD) with improvement in kidney function and proteinuria.

Discussion

This case of MIDD, occurring in a very young female, highlights the importance of complete serologic evaluation of individuals with new onset nephrotic syndrome, including SPEP and sFLC. MIDD is associated with multiple myeloma in up to 60% of cases but can occur in absence of hematological malignancy. Kidney is the major target organ in MIDD but about 35% of cases have extrarenal manifestations. Clone-directed chemotherapy with CyBorD or bendamustine is the treatment of choice. The sensitivity of abnormal serum FLC is very high in MIDD, hence is an important marker for both suspicion of disease and remission.

Clockwise: IF IgG, LM – Silver - Diffuse mesangial and endocapillary proliferation with lobular accentuation, IF IgG lambda, EM X4000 – Powdery deposits along TBM