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Master Thesis

Prise en charge des syndromes de Fanconi secondaires à une immunoglobuline monoclonale

Abstract : Adult-acquired Fanconi syndrome (FS) is a rare complication of monoclonal gammopathy. It is characterized by urinary leak of phosphorus, uric acid and glucose. We retrospectively reviewed 21 patients diagnosed with adult-acquired FS between 1988 and 2012 in seven French centers.
At diagnosis most patients had smoldering multiple myeloma (47%) or monoclonal gammopathy of undetermined significance (28%). They were usually referred for osteomalacia (30%) or chronic renal failure (55%). Every patient had tubular dysfunction and median creatinine level was 141 μmol/L (range 94-298). 19/21 patients had a renal biopsy performed, 40% showing characteristic crystal inclusions.
18 patients received chemotherapy. The main indication for treatment was renal preservation. High dose melphalan followed by autologous stem cell transplantation was administrated in nine cases, without major complications. Six patients received bortezomib based therapy, with frequent grade III neuropathy. Imids were usually second line treatment, with acceptable safety and efficiency. Hematological response to treatment was satisfying. Six patients (33%) had an improvement of tubular disorder; every patient had a stabilization of creatinine level until free light chain (FLC) elevation.
In conclusion, chemotherapy treatment may be proposed to patients with Fanconi syndrome if they are fit with a prolonged life expectancy. Benefice on renal function is probably better in case of early treatment.
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Master Thesis
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Submitted on : Monday, May 18, 2015 - 10:10:42 AM
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  • HAL Id : dumas-01148381, version 1


Marguerite Vignon. Prise en charge des syndromes de Fanconi secondaires à une immunoglobuline monoclonale. Médecine humaine et pathologie. 2013. ⟨dumas-01148381⟩



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