High-dose melphalan followed by autologous stem cell support in primary systemic AL amyloidosis with multiple organ involvement.
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.
Shimojima
Yasuhiro
Y
Third Department of Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Matsuda
Masayuki
M
Ishii
Wataru
W
Koyama
Jun
J
Yamamoto
Kanji
K
Shimodaira
Shigetaka
S
Sakashita
Kazuo
K
Koike
Kenichi
K
Ikeda
Shu-ichi
S
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
D000682:Amyloid; D018906:Antineoplastic Agents, Alkylating; D007147:Immunoglobulin Light Chains; D045265:Natriuretic Peptides; D008558:Melphalan