Hematologic and renal improvement of monoclonal immunoglobulin deposition disease after treatment with bortezomib-based regimens

Abstract

Monoclonal immunoglobulin deposition disease (MIDD) is characterized by non-organized immunoglobulin-fragments along renal basement membranes with subsequent organ deterioration. Treatment is directed against the immunoglobulin-producing clone. We treated 18 MIDD patients with bortezomib-based regimens (12 received bortezomib-dexamethasone, 6 bortezomib-dexamethasone with cyclophosphamide). Eleven (61%) patients achieved a hematologic response, but only 6 (33.3%) reached to a complete (CR) or very good partial response (VGPR). Regarding renal outcomes 77.8 and 55.6% had ≥30 and ≥50% reduction of proteinuria, respectively, but 33.3% ended up in end-stage renal disease (ESRD). Among patients with CR or VGPR, median eGFR improvement was 7.7 ml/min/1.73 m² and none progressed to ESRD, but no significant renal recovery was observed in patients achieving a partial response or less, with 50% progressing to dialysis. Pretreatment eGFR seems to influence renal prognosis. Bortezomib-based treatment is considered an effective approach in MIDD and reaching to a deep hematologic response (≥VGPR) conditionally controls further renal declining.

Keywords:

• MIDD
• bortezomib
• renal response
• proteinuria
• ESRD

Acknowledgments

All named authors have read the manuscript and have agreed to submit the article to Leukemia and Lymphoma in its present form. All those named as authors have made a sufficient contribution to the design, execution, and analysis of the article.

Potential conflict of interest

Disclosure forms provided by the authors are available with the full text of this article at (http://dx.doi.org/10.1080/10428194.2016.1267349).