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Kidney Int. 2015 Nov;88(5):1135-43. doi: 10.1038/ki.2015.201. Epub 2015 Jul 15.

Bortezomib produces high hematological response rates with prolonged renal survival in monoclonal immunoglobulin deposition disease.

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Department of Nephrology, Necker Hospital, Paris, France.
Department of Hematology, CHU Amiens, Amiens, France.
Department of Nephrology, CHU Poitiers, Centre National de Référence Maladies Rares: Amylose AL et Autres Maladies à Dépôts d'Immunoglobulines Monoclonales, Poitiers, France.
Department of Immunology and Hematology, Saint Louis Hospital, Paris, France.
Department of Hematology, CHU Limoges, Centre National de Référence Maladies Rares: Amylose AL et Autres Maladies à Dépôts d'Immunoglobulines Monoclonales, Limoges, France.
Department of Biostatistics and Informatics, Saint Louis Hospital, Paris, France.


Monoclonal immunoglobulin deposition disease (MIDD) is a rare complication of plasma cell disorders, defined by linear Congo red-negative deposits of monoclonal light chain, heavy chain, or both along basement membranes. While renal involvement is prominent, treatment strategies, such as the impact of novel anti-myeloma agents, remain poorly defined. Here we retrospectively studied 49 patients with MIDD who received a median of 4.5 cycles of intravenous bortezomib plus dexamethasone. Of these, 25 received no additional treatment, 18 also received cyclophosphamide, while 6 also received thalidomide or lenalidomide. The hematological diagnoses identified 38 patients with monoclonal gammopathy of renal significance, 10 with symptomatic multiple myeloma, and 1 with Waldenstrom macroglobulinemia. The overall hematologic response rate, based on the difference between involved and uninvolved serum-free light chains (dFLCs), was 91%. After median follow-up of 54 months, 5 patients died and 10 had reached end-stage renal disease. Renal response was achieved in 26 patients, with a 35% increase in median eGFR and an 86% decrease in median 24-h proteinuria. Predictive factors were pre-treatment eGFR over 30 ml/min per 1.73 m(2) and post-treatment dFLC under 40 mg/l; the latter was the sole predictive factor of renal response by multivariable analysis. Thus, bortezomib-based therapy is a promising treatment strategy in MIDD, mainly when used early in the disease course. dFLC response is a favorable prognostic factor for renal survival.

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