title: [Clinical analysis of autologous stem cell transplantation for nine cases of cardiac amyloidosis].
abstract: Long-term survival remains poor for patients with cardiac amyloidosis. High-dose melphalan (MEL) with stem cell transplantation (HDM/SCT) is an effective treatment for AL amyloidosis, but patients with cardiac involvement are ineligible because of high therapy-related mortality. Here we report detailed HDM/SCT outcomes of 9 patients with cardiac failure. Their median age was 56 years (range, 45∼66). After a median follow-up of 15 months (range 9∼32), three died of multiorgan failure within the early phase after HDM/SCT, and the other six including poor risk patients are alive at present. Their symptoms of cardiac decompensation have improved. Decreases in interventricular septum thickness were confirmed in 4 patients 6∼12 months after HDM/SCT by echocardiography. One-year overall survival rate was 67%, longer than previously reported rates. HDM/SCT may lead to improvements in quality of life and extended survival in cardiac amyloidosis patients. Meanwhile, the median dosage of MEL in our procedure was 103 mg/m(2) (range 68∼180), less than the recommended dose, and patients were maintained on miscellaneous therapies. Further studies are required to clarify an effective MEL dose and to refine selection criteria for patients undergoing HDM/SCT.
authors.affiliation: Department of Hematology, Japanese Red Cross Medical Center.
authors.forename: Mizuki
authors.initials: M
authors.lastname: Ogura
authors.forename: Rieko
authors.initials: R
authors.lastname: Sekine
authors.forename: Sayuri
authors.initials: S
authors.lastname: Nishiyama
authors.forename: Yu
authors.initials: Y
authors.lastname: Abe
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Iizuka
authors.forename: Sayaka
authors.initials: S
authors.lastname: Kusaka
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Nakagawa
authors.forename: Kenshi
authors.initials: K
authors.lastname: Suzuki
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D009202:Cardiomyopathies / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D006333:Heart Failure / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D006334:Heart Function Tests; D006801:Humans; D008297:Male; D008558:Melphalan / Q000008:administration & dosage*; D008875:Middle Aged; D036102:Peripheral Blood Stem Cell Transplantation; D012189:Retrospective Studies; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D008558:Melphalan