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Kidney Week

Abstract: SA-PO181

Monoclonal Immunoglobulin Deposition Disease: Experience in a Single Institution

Session Information

  • Onco-Nephrology: Clinical
    November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Yang, Yihe, Northwell Health, Bayside, New York, United States
  • Parikh, Rushang, Northwell Health, Bayside, New York, United States
  • Haghi, Nina, Northwell Health, Bayside, New York, United States
  • Sharma, Purva D., Northwell Health, Bayside, New York, United States
  • Wanchoo, Rimda, Northwell Health, Bayside, New York, United States
  • Jhaveri, Kenar D., Northwell Health, Bayside, New York, United States
  • Bijol, Vanesa, Northwell Health, Bayside, New York, United States
Background

Recently described proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID) has become of interest as it typically has clinically significant course, with a membranoproliferative (MPGN) pattern on light microscope, and selective glomerular deposition of an entire monoclonal immunoglobulin by IF. There is often no hematologic malignancy to explain the findings, leading to therapeutic difficulties.

Methods

We reviewed our MIDD cases (7/1/2017-5/1/2019).

Results

The prevalence of MIDD is 3% (28/860), including entire monoclonal immunoglobulin or light and heavy chain deposition disease (L&HCDD, n=12), renal light chain (AL) amyloidosis (n=13), κ light chain deposition disease (KCDD, n=1), and γ heavy chain deposition disease (HCDD, n=2). The L&HCDD group had 9 cases of monoclonal IgG deposition disease (PGNMID), with a predominant MPGN pattern, and 3 cases of monoclonal IgA DD, with a predominant mesangial glomerular involvement. Bone marrow (BM) was biopsied in 6/12 of the L&HCDD cases; 3 showed abnormalities. Serologically, 6/12 had no M-spike; 1 case with M-spike had negative BM workup; 1 case had an M-spike without BM biopsy; and 1 case had no serological workup. 7/9 PGNMID cases received bortezomib based treatment. Pre- and post-treatment proteinuria was 8.76±9.95 and 1.75±2.06; creatinine was 1.97±0.64 and 1.94±0.81mg/dL. Other MIDD cases had a clear connection with BM malignancy: multiple myeloma in 9/13 amyloid cases and all HCDD and KLCDD cases;1 case had a negative BM evaluation but a IgG-λ circulating paraprotein.

Conclusion

L&HCDD is almost as common as AL amyloidosis, and much more common than LCDD or HCDD. Despite not identifying a monoclonal disease in many of our PGNMID cases, bortezomib based treatment resulted in good renal outcomes.