Clinical benefits of bortezomib-containing regimens for newly diagnosed AL amyloidosis with severe cardiac impairment.
Cardiac amyloid light-chain amyloidosis (AL amyloidosis) is a rare disease with a very poor prognosis, associated with plasma cell dyscrasias such as monoclonal gammopathy of undetermined significance and multiple myeloma. Though bortezomib-containing regimens have achieved high hematologic response rates, there are still few reports describing the outcomes of Japanese patients. Six patients with severe cardiac AL amyloidosis were treated with bortezomib-containing regimens. Involved free light chain (iFLC) decreased immediately in most of these cases. However, the condition of heart failure and N-terminal pro-B-type natriuretic peptide (NT-proBNP) worsened in the early phase of this treatment and then improved several months later. At 29 months, the median duration of follow-up (2-47months), all patients remain alive except one who died of sudden cardiac arrest. Bortezomib-containing regimens are considered to be among the effective treatments for severe cardiac AL amyloidosis.
Department of Hematology, Juntendo University School of Medicine.
Yutaka
Y
Tsukune
Yuriko
Y
Yahata
Makoto
M
Sasaki
Makoto
M
Hiki
Miyuki
M
Tsutsui
Yasuharu
Y
Hamano
Seigo
S
Itoh
Tetsuro
T
Miyazaki
Tomotaka
T
Dohi
Masaki
M
Maruyama
Akihiko
A
Gotoh
Norio
N
Komatsu
D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000188:drug therapy*; D000069286:Bortezomib / Q000627:therapeutic use*; D005260:Female; D006331:Heart Diseases / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
D000069286:Bortezomib