caa a22 4500 44588326X CHVBK 20180317145551.0 cr unu---uuuuu 170323e20110801xx s 000 0 eng 10.1007/s10157-011-0426-0 doi (NATIONALLICENCE)springer-10.1007/s10157-011-0426-0 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 [Elektronische Daten] [Junichi Hoshino, Yoshifumi Ubara, Naoki Sawa, Keiichi Sumida, Rikako Hiramatsu, Eiko Hasegawa, Tatsuya Suwabe, Noriko Hayami, Masayuki Yamanouchi, Fumi Takemoto, Shuichi Taniguchi, Kenmei Takaichi] 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, log-rank 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 log-rank 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. Japanese Society of Nephrology, 2011 Systemic AL amyloidosis nationallicence Treatment nationallicence Prognosis nationallicence Melphalan nationallicence VAD nationallicence HDM : High-dose melphalan and autologous stem cell transplantation nationallicence MP : Melphalan+prednisolone nationallicence VAD : Vincristine+adriamycin+dexamethasone nationallicence MD : Melphalan+dexamethasone nationallicence LVEF : Left ventricular ejection fraction nationallicence LV : Left ventricle nationallicence PVF : Pulmonary venous flow nationallicence TMF : Transmitral flow nationallicence ALP : Alkaline phosphatase nationallicence BNP : Brain natriuretic peptide nationallicence SD : Standard deviation nationallicence Alb : Serum albumin nationallicence Cr : Serum creatinine nationallicence GI : Gastrointestinal nationallicence IQR : Interquartile range nationallicence RR : Relative risk nationallicence CI : Confidence interval nationallicence Hoshino Junichi Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Ubara Yoshifumi Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Sawa Naoki Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Sumida Keiichi Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Hiramatsu Rikako Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Hasegawa Eiko Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Suwabe Tatsuya Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Hayami Noriko Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Yamanouchi Masayuki Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Takemoto Fumi Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Taniguchi Shuichi Department of Hematology, Toranomon Hospital, Tokyo, Japan aut Takaichi Kenmei Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut Clinical and Experimental Nephrology Springer Japan 15/4(2011-08-01), 486-492 1342-1751 15:4<486 2011 15 10157 https://doi.org/10.1007/s10157-011-0426-0 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10157-011-0426-0 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Hoshino Junichi Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Ubara Yoshifumi Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Sawa Naoki Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Sumida Keiichi Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Hiramatsu Rikako Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Hasegawa Eiko Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Suwabe Tatsuya Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Hayami Noriko Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Yamanouchi Masayuki Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Takemoto Fumi Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 700 1- Taniguchi Shuichi Department of Hematology, Toranomon Hospital, Tokyo, Japan aut NATIONALLICENCE 700 1- Takaichi Kenmei Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato-ku, 105-8470, Tokyo, Japan aut NATIONALLICENCE 773 0- Clinical and Experimental Nephrology Springer Japan 15/4(2011-08-01), 486-492 1342-1751 15:4<486 2011 15 10157 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer