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Kidney Week

Abstract: SA-PO182

Outcomes of Renal Transplantation in Monoclonal Immunoglobulin Deposition Disease

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

  • Angel-Korman, Avital, Boston Medical Center, Boston, Massachusetts, United States
  • Sanchorawala, Vaishali, Boston University School of Medicine, Boston, Massachusetts, United States
  • Havasi, Andrea, Boston University Medical Center, Boston, Massachusetts, United States
Background

Renal involvement in monoclonal immunoglobulin deposition disease (MIDD) is close to 100%. Therapies have dramatically improved in the last 2 decades leading to deeper hematologic responses and longer disease-free survival, however renal organ survival is lagging behind and progression to end stage renal disease (ESRD) is frequent. Data on overall survival (OS) and renal transplant outcome in this patient population are limited.

Methods

We report the outcomes of 23 patients with MIDD of whom 6 patients underwent renal transplantation. All patients were followed in the Amyloidosis Center at Boston University School of Medicine between January 1989 and December 2018.

Results

At the time of diagnosis median age was 51.7 years, median eGFR was 22 mL/min/1.73m2 (range, 4-91) and median proteinuria was 3g (range, 0.8-12). At censor, 9 of the 23 patients (39%) were deceased. One, 5 and 10-year survival from diagnosis were 95%, 78% and 65%, respectively. Renal organ response was achieved only in 5 patients (22%) after a median time of 1 year (range, 0.9-1.2) from diagnosis. One, 5 and 10-year renal survival from diagnosis were 72%, 36% and 21%, respectively. Twelve patients (52%) reached ESRD. Fourteen of the 18 patients (78%) who received first line treatment with high dose melphalan/stem cell transplantation (HDM/SCT) or bortezomib achieved a very good partial response or complete response. In the renal transplant patient group (n=6), shortest survival from diagnosis was 13.7 and the longest was 27.7 years. Three patients were still alive with functioning grafts at censor: 1.7, 2.8 and 6.5 years after renal transplantation. Grafts were lost either due to disease recurrence after 0.3 and 3.8 years or as a result of patient’s death 20.3 years from renal transplantation. Median OS from diagnosis of patients who progressed to ESRD was significantly better for those who underwent renal transplantation vs. those who did not (19.8 vs. 8.3 years, p= 0.016). Median OS from dialysis initiation was also better in this group of patients; however the difference was not statistically significant (p=0.06).

Conclusion

Renal transplantation is a viable option for some patients with MIDD and these selected patients have improved OS.

Funding

  • Private Foundation Support