title: High-dose melphalan followed by autologous stem cell support in primary systemic AL amyloidosis with multiple organ involvement.
abstract: A patient with primary systemic AL amyloidosis achieved partial hematological response after 2 courses of VAD (vincristine, doxorubicin and dexamethasone) and subsequent high-dose melphalan followed by autologous peripheral blood stem cell transplantation despite involvement of multiple organs, including the heart. In this patient natriuretic peptides and free light chains in serum were useful as markers of cardiac involvement and therapeutic effects, respectively. When amyloidosis-related dysfunction is seen in multiple organs, this intensive chemotherapy might be a possible therapeutic option, although several modifications in the regimen and careful management are necessary.
authors.affiliation: Third Department of Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Shimojima
authors.forename: Masayuki
authors.initials: M
authors.lastname: Matsuda
authors.forename: Wataru
authors.initials: W
authors.lastname: Ishii
authors.forename: Jun
authors.initials: J
authors.lastname: Koyama
authors.forename: Kanji
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Shigetaka
authors.initials: S
authors.lastname: Shimodaira
authors.forename: Kazuo
authors.initials: K
authors.lastname: Sakashita
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use*; D001707:Biopsy, Needle; D001853:Bone Marrow / Q000473:pathology; D004452:Echocardiography; D005500:Follow-Up Studies; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood; D008297:Male; D008558:Melphalan / Q000627:therapeutic use*; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D045265:Natriuretic Peptides / Q000097:blood; D036102:Peripheral Blood Stem Cell Transplantation; D013363:Submandibular Gland / Q000378:metabolism / Q000473:pathology; D013997:Time Factors; D014059:Tongue / Q000378:metabolism / Q000473:pathology; D014182:Transplantation, Autologous
chemical_list: D000682:Amyloid; D018906:Antineoplastic Agents, Alkylating; D007147:Immunoglobulin Light Chains; D045265:Natriuretic Peptides; D008558:Melphalan