Current anti-myeloma therapies in renal manifestations of monoclonal light chain-associated Fanconi syndrome

A retrospective series of 49 patients

M. Vignon, V. Javaugue, Mariam P Alexander, K. El-Karoui, A. Karras, D. Roos-Weil, B. Royer, B. Asli, B. Knebelmann, G. Touchard, A. Jaccard, B. Arnulf, F. Bridoux, N. Leung, J. P. Fermand

Research output: Contribution to journalArticle

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Abstract

We retrospectively reviewed 49 patients with light chain (LC) Fanconi syndrome (FS). Patients presented with chronic kidney disease (median estimated glomerular filtration rate (eGFR) of 33 ml/min/1.73 m 2) and tubular proteinuria. All patients tested had elevated fractional excretion of phosphate, uric acid, generalized aminoaciduria and/or normoglycemic glycosuria. Thirty-eight patients had monoclonal gammopathy of renal significance and eleven patients had an overt hematological malignancy. The monoclonal LC isotype was kappa in 46/49 cases. Kidney biopsy in 39 patients showed various proximal tubular lesions and characteristic LC intracytoplasmic crystalline inclusions in 24 patients. Forty-two patients received chemotherapy. Patients with plasma cell proliferation (n=38) received bortezomib-based regimens (n=11), immunomodulatory agents (n=7) or alkylating agents (n=6). High-dose melphalan (HDM) followed by autologous stem cell transplantation was performed in 14 patients. Hematological response was obtained in 90% of evaluable patients, assessed on serum free light chains (FLC). GFR remained stable as long as hematological response was maintained and declined when serum FLC level rebounded. Improvement in proximal tubule function occurred in 13 patients. In patients with LC-associated FS, chemotherapy using HDM and/or new generation anti-myeloma agents can stabilize renal function and improve proximal tubule function. Serum FLC should be used to assess the hematological response, related to renal outcome.

Original languageEnglish (US)
Pages (from-to)123-129
Number of pages7
JournalLeukemia
Volume31
Issue number1
DOIs
StatePublished - Jan 1 2017

Fingerprint

Fanconi Syndrome
Kidney
Light
Therapeutics
Melphalan
Serum
Glycosuria
Drug Therapy
Paraproteinemias
Alkylating Agents
Stem Cell Transplantation
Hematologic Neoplasms
Plasma Cells
Uric Acid
Glomerular Filtration Rate
Chronic Renal Insufficiency
Proteinuria

ASJC Scopus subject areas

  • Hematology
  • Cancer Research
  • Anesthesiology and Pain Medicine

Cite this

Current anti-myeloma therapies in renal manifestations of monoclonal light chain-associated Fanconi syndrome : A retrospective series of 49 patients. / Vignon, M.; Javaugue, V.; Alexander, Mariam P; El-Karoui, K.; Karras, A.; Roos-Weil, D.; Royer, B.; Asli, B.; Knebelmann, B.; Touchard, G.; Jaccard, A.; Arnulf, B.; Bridoux, F.; Leung, N.; Fermand, J. P.

In: Leukemia, Vol. 31, No. 1, 01.01.2017, p. 123-129.

Research output: Contribution to journalArticle

Vignon, M, Javaugue, V, Alexander, MP, El-Karoui, K, Karras, A, Roos-Weil, D, Royer, B, Asli, B, Knebelmann, B, Touchard, G, Jaccard, A, Arnulf, B, Bridoux, F, Leung, N & Fermand, JP 2017, 'Current anti-myeloma therapies in renal manifestations of monoclonal light chain-associated Fanconi syndrome: A retrospective series of 49 patients', Leukemia, vol. 31, no. 1, pp. 123-129. https://doi.org/10.1038/leu.2016.195
Vignon, M. ; Javaugue, V. ; Alexander, Mariam P ; El-Karoui, K. ; Karras, A. ; Roos-Weil, D. ; Royer, B. ; Asli, B. ; Knebelmann, B. ; Touchard, G. ; Jaccard, A. ; Arnulf, B. ; Bridoux, F. ; Leung, N. ; Fermand, J. P. / Current anti-myeloma therapies in renal manifestations of monoclonal light chain-associated Fanconi syndrome : A retrospective series of 49 patients. In: Leukemia. 2017 ; Vol. 31, No. 1. pp. 123-129.
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