title: Successful Treatment of Amyloid Light-chain Amyloidosis in a Charcot-Marie-Tooth Disease Patient with Lenalidomide, Cyclophosphamide, and Dexamethasone.
abstract: 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.
authors.affiliation: Departments of Hematology, Rheumatology, and Infectious Disease, Kumamoto University Graduate School of Medicine, Japan.
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Kikukawa
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Hata
authors.forename: Mitsuharu
authors.initials: M
authors.lastname: Ueda
authors.forename: Taro
authors.initials: T
authors.lastname: Yamashita
authors.forename: Singo
authors.initials: S
authors.lastname: Nasu
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Ide
authors.forename: Shikiko
authors.initials: S
authors.lastname: Ueno
authors.forename: Yukio
authors.initials: Y
authors.lastname: Ando
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Mitsuya
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Okuno
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000188:drug therapy* / Q000209:etiology*; D002607:Charcot-Marie-Tooth Disease / Q000150:complications*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology; D000075363:Immunoglobulin Light-chain Amyloidosis; D007146:Immunoglobulin lambda-Chains / Q000276:immunology; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000077269:Lenalidomide; D012074:Remission Induction; D013792:Thalidomide / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D007147:Immunoglobulin Light Chains; D007146:Immunoglobulin lambda-Chains; D007166:Immunosuppressive Agents; D013792:Thalidomide; D003907:Dexamethasone; D003520:Cyclophosphamide; D000077269:Lenalidomide