Bortezomib-based chemotherapy can improve renal and tubular functions in patients with light chain-associated Fanconi syndrome

  • Xia Wu
  • Lu Zhang
  • Jun Feng
  • Yue-ying Mao
  • Xin-xin Cao
  • Dao-bin Zhou
  • Jian LiEmail author
Original Article


Light chain-associated Fanconi syndrome (LCFS) is a disorder of renal proximal tubule due to immunoglobulin light chains. Cases of LCFS are rare and mostly sporadically reported, and treatment of this entity is still controversial. This single-center retrospective study included 22 patients diagnosed with LCFS in Peking Union Medical College Hospital. Monoclonal gammopathy of undetermined significance was diagnosed in 13 patients, and overt multiple myeloma in six patients, with two smoldering myeloma and one Waldenstrom macroglobulinemia. Light chain was mostly kappa type (90.9%). Baseline median estimated glomerular filtration rate was 66 (13–126) ml/min/1.73 m2, with one patient presented as end-stage renal disease. After a median follow-up of 37 months, three patients died. Twelve patients were treated with chemotherapy, including 7 with bortezomib-based regimens. Renal function was significantly improved in the group of patients who received chemotherapy (p = 0.026). Compared with other chemotherapy regimens, patients with bortezomib-based treatment had a better hematological response (p = 0.027) as well as a better proximal tubule outcome (p = 0.015). Chemotherapy likely outweighs supportive treatment in patients with LCFS. Bortezomib-based regimen seems to be a safe first-line therapy for management of those patients.


Light chain-associated Fanconi syndrome Bortezomib Plasma cell Renal function Tubular function 


Authors’ contributions

XW designed and performed the study and took the lead in writing the manuscript. LZ, JF, YM, XC, and DZ performed the study and revised the manuscript. JL conceived the original idea, designed and performed the study, and revised the manuscript.


The authors thank the patients and their families. Institutional research funding was provided by the Peking Union Medical College New Star (2011, to LJ), the CAMS Innovation Fund for Medical Sciences (Grant No. 2016-12M-1-002), and The National Key Research and Development Program of China (Grant No. 2016YFC0901503).

Compliance with ethical standards

A written informed consent was obtained from each patient. This study was approved by the Ethics Committee of the hospital.

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

277_2018_3572_MOESM1_ESM.pdf (36 kb)
ESM 1 (PDF 35 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Xia Wu
    • 1
  • Lu Zhang
    • 1
  • Jun Feng
    • 1
  • Yue-ying Mao
    • 1
  • Xin-xin Cao
    • 1
  • Dao-bin Zhou
    • 1
  • Jian Li
    • 1
    Email author
  1. 1.Department of Hematology, Peking Union Medical College HospitalPeking Union Medical College and Chinese Academy of Medical SciencesBeijingPeople’s Republic of China

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