title: Nephrotic syndrome due to primary AL amyloidosis, successfully treated with VAD and subsequent high-dose melphalan followed by autologous peripheral blood stem cell transplantation.
abstract: Primary AL amyloidosis involves vital organs from the early phase of illness, resulting in a poor prognosis. We report a patient with nephrotic syndrome due to this type of amyloidosis, who was successfully treated with two courses of VAD (vincristine, doxorubicin and dexamethasone) and subsequent high-dose melphalan (140 mg/m2) with autologous stem cell support. Following the serial chemotherapy his proteinuria improved, and M protein became undetectable in both serum and urine. To avoid the progression of primary AL amyloidosis, intensive chemotherapy should be actively used when the general status and vital organ functions are well preserved.
authors.affiliation: Third Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621.
authors.forename: Takahisa
authors.initials: T
authors.lastname: Gono
authors.forename: Masayuki
authors.initials: M
authors.lastname: Matsuda
authors.forename: Naoko
authors.initials: N
authors.lastname: Dohi
authors.forename: Kenichi
authors.initials: K
authors.lastname: Hoshi
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Tada
authors.forename: Kazuo
authors.initials: K
authors.lastname: Sakashita
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
authors.forename: Masatsugu
authors.initials: M
authors.lastname: Aizawa
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications* / Q000473:pathology / Q000628:therapy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D014182:Transplantation, Autologous
chemical_list: D000682:Amyloid; D005047:Etoposide; D004317:Doxorubicin; D008558:Melphalan