title: Therapeutic outcome of cyclic VAD (vincristine, doxorubicin and dexamethasone) therapy in primary systemic AL amyloidosis patients.
abstract: OBJECTIVE
  Intensive chemotherapy targeting plasma cell dyscrasia has been recently employed for the treatment of primary systemic AL amyloidosis. We prospectively studied the clinical usefulness of cyclic VAD (vincristine, doxorubicin and dexamethasone) in patients with primary systemic AL amyloidosis who were ineligible for high-dose melphalan with autologous stem cell support.
  
  
  METHODS
  Eight patients (mean age, 60.4+/-8.8 years) were treated with cyclic VAD until the disappearance of M-protein from both serum and urine. Of these, seven showed nephrotic syndrome before the start of VAD irrespective of a decrease in creatinine clearance. Serial follow-up studies after VAD evaluated hematological status and organ function.
  
  
  RESULTS
  Four patients (50%) showed a marked decrease in abnormal plasma cells in the bone marrow and normalized kappa/lambda ratios of serum free light chain in conjunction with disappearance of M-protein after 1 to 3 courses of VAD. There were no serious adverse events, and nephrotic syndrome gradually improved with no hematological relapse in the follow-up period of 3 to 5 years. The remaining 4 patients showed worsening of congestive heart failure and/or systemic edema ascribable to dexamethasone, resulting in cessation of cyclic VAD before disappearance of M-protein. All of these patients died of multiple organ failure or required permanent hemodialysis within 1 year after the start of cyclic VAD.
  
  
  CONCLUSIONS
  Cyclic VAD is a potent therapeutic option in primary systemic AL amyloidosis, but in patients with renal or cardiac dysfunction careful management for adverse events, especially body fluid retention, is necessary.
authors.affiliation: Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine.
authors.forename: Ko-Ichi
authors.initials: K
authors.lastname: Tazawa
authors.forename: Masayuki
authors.initials: M
authors.lastname: Matsuda
authors.forename: Takuhiro
authors.initials: T
authors.lastname: Yoshida
authors.forename: Takahisa
authors.initials: T
authors.lastname: Gono
authors.forename: Nagaaki
authors.initials: N
authors.lastname: Katoh
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Shimojima
authors.forename: Wataru
authors.initials: W
authors.lastname: Ishii
authors.forename: Tomohisa
authors.initials: T
authors.lastname: Fushimi
authors.forename: Jun
authors.initials: J
authors.lastname: Koyama
authors.forename: Shu-Ichi
authors.initials: S
authors.lastname: Ikeda