Successful Treatment of Amyloid Light-chain Amyloidosis in a Charcot-Marie-Tooth Disease Patient with Lenalidomide, Cyclophosphamide, and Dexamethasone

Intern Med. 2016;55(18):2707-12. doi: 10.2169/internalmedicine.55.5815. Epub 2016 Sep 15.


A 70-year-old woman with Charcot-Marie-Tooth disease (CMT) suffered from nephrotic syndrome and a renal biopsy revealed non-AA amyloid depositions that contained immunoglobulin light chain λ. Her serum λ free LC was elevated to 80.8 mg/L and she was diagnosed with primary amyloid light-chain (AL) amyloidosis. She was subsequently treated with lenalidomide, cyclophosphamide, and dexamethasone (RCD). After 14 cycles of RCD, she achieved complete remission. Her serum albumin levels gradually normalized to 3.1 g/dL. No exacerbation of neurologic symptoms related to CMT was observed. Thus, RCD may be a well-tolerated and effective regimen for treating AL amyloidosis in patients with CMT disease.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Amyloidosis / drug therapy*
  • Amyloidosis / etiology*
  • Charcot-Marie-Tooth Disease / complications*
  • Cyclophosphamide / therapeutic use
  • Dexamethasone / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunoglobulin Light Chains / immunology
  • Immunoglobulin Light-chain Amyloidosis
  • Immunoglobulin lambda-Chains / immunology
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / therapeutic use*
  • Lenalidomide
  • Remission Induction
  • Thalidomide / analogs & derivatives
  • Thalidomide / therapeutic use


  • Immunoglobulin Light Chains
  • Immunoglobulin lambda-Chains
  • Immunosuppressive Agents
  • Thalidomide
  • Dexamethasone
  • Cyclophosphamide
  • Lenalidomide