title: Improvement of cardiac diastolic function and prognosis after autologous peripheral blood stem cell transplantation in AL cardiac amyloidosis.
abstract: AL amyloidosis is a disease in which immunoglobulin L chain is deposited in multiple organs, and the prognosis of cardiac amyloidosis is extremely poor. Although several treatments based on that for multiple myeloma, have been performed, there is no clear evidence that cardiac function is improved. We report a case of AL cardiac amyloidosis with moderate cardiac dysfunction for which we performed autologous peripheral blood stem cell transplantation (auto-PBSCT) in combination with high-dose melphalan therapy. This treatment resulted in significant improvement in cardiac function and good prognosis for about 3.5 years after the diagnosis. Therefore, auto-PBSCT is a possible option as up-front therapy for AL cardiac amyloidosis.
authors.affiliation: Department of Medicine and Bioregulatory Sciences, Institute of Health Biosciences, The University of Tokushima Graduate School. syagi@clin.med.tokushima-u.ac.jp
authors.forename: Shusuke
authors.initials: S
authors.lastname: Yagi
authors.forename: Masashi
authors.initials: M
authors.lastname: Akaike
authors.forename: Shuji
authors.initials: S
authors.lastname: Ozaki
authors.forename: Chikako
authors.initials: C
authors.lastname: Moriya
authors.forename: Kyoko
authors.initials: K
authors.lastname: Takeuchi
authors.forename: Tomoko
authors.initials: T
authors.lastname: Hara
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Fujimura
authors.forename: Yuka
authors.initials: Y
authors.lastname: Sumitomo
authors.forename: Takashi
authors.initials: T
authors.lastname: Iwase
authors.forename: Yasumasa
authors.initials: Y
authors.lastname: Ikeda
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Aihara
authors.forename: Takehiko
authors.initials: T
authors.lastname: Kimura
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nishiuchi
authors.forename: Masahiro
authors.initials: M
authors.lastname: Abe
authors.forename: Toshio
authors.initials: T
authors.lastname: Matsumoto
mesh_terms: D000686:Amyloidosis / Q000503:physiopathology / Q000628:therapy*; D009202:Cardiomyopathies / Q000503:physiopathology / Q000628:therapy*; D003131:Combined Modality Therapy; D005260:Female; D006321:Heart / Q000503:physiopathology; D006801:Humans; D008558:Melphalan / Q000008:administration & dosage; D008875:Middle Aged; D036102:Peripheral Blood Stem Cell Transplantation; D011379:Prognosis
chemical_list: D008558:Melphalan