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Master Thesis

Efficacité du bortezomib dans la maladie des dépôts d’immunoglobulines monoclonales de type Randall

Abstract : Monoclonal immunoglobulin (Ig) deposition disease (MIDD) is a rare complication of plasma cell disorders, defined by linear Congo red-negative deposits of monoclonal light chain (LCDD), heavy chain (HCDD) or both (LHCDD) along basement membranes. Renal involvement is prominent. Treatment strategies, including the impact of novel anti-myeloma agents remain poorly defined. We retrospectively studied 49 MIDD patients who received a median of 4.5 cycles of intravenous Bortezomib (1.3mg/m2) +dexamethasone (BD) (n=25), BD+cyclophosphamide (n=18), or BD+thalidomide or lenalidomide (n=6). The hematological diagnosis was monoclonal gammopathy of renal significance (n=38), symptomatic multiple myeloma (n=10), and Waldenström macroglobulinemia (n=1). The overall hematologic response rate, based on the difference between involved and uninvolved serum free light chains (dFLC), was 91%. After median follow-up of 54 months, 5 patients had died and 2 had reached end-stage renal disease. Renal response was achieved in 26 patients (53%), with 35% increase in median eGFR and 86% decrease in median 24-hour proteinuria. Predictive factors of renal response were pre-treatment eGFR>30mL/min/1.73m2 and post-treatment dFLC<40 mg/L. By multivariate analysis, achievement of post-treatment dFLC<40 mg/L was the sole predictive factor of renal response (OR= 1.04, 95CI: 0.99 - 1.09). In MIDD, bortezomib-based therapy is safe and efficient when introduced early after diagnosis. dFLC response is a favorable prognostic factor for renal survival.
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Master Thesis
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Submitted on : Friday, October 23, 2015 - 3:41:53 PM
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  • HAL Id : dumas-01219942, version 1


Camille Cohen. Efficacité du bortezomib dans la maladie des dépôts d’immunoglobulines monoclonales de type Randall. Médecine humaine et pathologie. 2015. ⟨dumas-01219942⟩



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