Primary AL amyloid polyneuropathy successfully treated with high-dose melphalan followed by autologous stem cell transplantation.
We report 2 patients with polyneuropathy associated with amyloid derived from light chains (AL) who were treated successfully with high-dose melphalan followed by autologous peripheral blood stem cell transplantation (HDM/SCT). Neuropathic symptoms improved in conjunction with normalization of serum-free light chains. In addition to amyloid deposits in tissues, an amyloidogenic light chain itself produced by abnormal plasma cells might be harmful to peripheral nerve function, and thus HDM/SCT seems to be a promising therapy for primary AL amyloid polyneuropathy.
Katoh
Nagaaki
N
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan.
Matsuda
Masayuki
M
Yoshida
Takuhiro
T
Yazaki
Masahide
M
Morita
Hiroshi
H
Sakashita
Kazuo
K
Ikeda
Shu-Ichi
S
D000328:Adult; D017772:Amyloid Neuropathies / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery; D001706:Biopsy; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D004576:Electromyography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008558:Melphalan / Q000008:administration & dosage*; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D019653:Myeloablative Agonists / Q000008:administration & dosage; D033581:Stem Cell Transplantation / Q000379:methods*; D014182:Transplantation, Autologous
D019653:Myeloablative Agonists; D008558:Melphalan