How to treat patients with systemic amyloid light chain amyloidosis? Comparison of high-dose melphalan, low-dose chemotherapy and no chemotherapy in patients with or without cardiac amyloidosis
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Junichi Hoshino Yoshifumi Ubara Naoki Sawa Keiichi Sumida Rikako Hiramatsu Eiko Hasegawa Tatsuya Suwabe Noriko Hayami Masayuki Yamanou
- چاپ و سال / کشور: 2011
Description
Background High-dose melphalan and autologous stem cell transplantation (HDM) is an effective treatment for systemic amyloid light chain (AL) amyloidosis but the eligibility criteria exclude many patients with this disorder. The aim of this study was to determine appropriate treatment strategies for systemic AL amyloidosis according to each patient’s clinical condition in Japan. Methods Historical cohort study. Fifty-three patients with systemic AL amyloidosis (those with malignancies were excluded) were treated in our hospital with HDM (15 patients), melphalan ? prednisolone (MP) (17 patients), vincristine ? adriamycin ? dexamethasone (VAD) (11 patients), or supportive treatment (no chemotherapy, 10 patients). We compared the survival rates among these treatment groups. Results Mean survival was significantly longer in the HDM group than in the other three groups (P\0.01, logrank test). This trend remained the same when patients were divided into those with and without cardiac amyloid involvement. Furthermore, in patients with heart involvement, survival in the VAD therapy group was significantly inferior to that in the MP therapy group (P\0.01 by logrank test). Significant factors related to the survival rate included the presence or absence of heart involvement and treatment modality. Conclusions HDM should be considered the treatment of choice in eligible patients with systemic AL amyloidosis even in the presence of cardiac amyloidosis. If HDM is not eligible, indications for VAD therapy should be carefully evaluated in patients with cardiac amyloidosis
Clin Exp N Received: 28 July 2010 / Accepted: 16 February 2011 Japanese Society of Nephrology 2011ephrol
