ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2017, Vol. 26 ›› Issue (3): 201-205.DOI: 10.3969/cndt.j.issn.1006-298X.2017.03.001

• 论文 •    下一篇

来那度胺联合地塞米松治疗复发难治性轻链淀粉样变性的疗效及安全性

  

  • 出版日期:2017-06-28 发布日期:2017-06-29

Efficacy and safety of lenalidomide with dexamethasone in relapse/refractory lightchain amyloidosis

  • Online:2017-06-28 Published:2017-06-29

摘要:

目的:观察来那度胺联合地塞米松(LD)治疗复发难治性轻链淀粉样变性(AL型)的临床疗效及安全性。
方法:收集2012年12月至2015年11月在南京总医院国家肾脏疾病临床医学研究中心接受LD治疗的复发难治性AL淀粉样变性患者的临床资料。回顾性分析血液学、器官反应情况及不良反应。
结果:共10例患者纳入研究,其中男性7例、女性3例,中位年龄为645(568,688)岁。7例患者纳入疗效分析,3例患者获得血液学应,其中1例获得完全缓解。7例患者均未获得肾脏反应,2例获得心脏反应。所有患者均存活,无血液学进展,2例心脏进展,1例进入终末期肾病。无一例发生血液学进展。3/4级不良反应发生率30%。
结论:LD方案作为复发难治性AL淀粉样变性患者的挽救治疗有一定的疗效且安全性较高,严重不良反应发生率较低。

关键词: 轻链淀粉样变性, 来那度胺, 疗效, 不良反应

Abstract:

Objective:To observe the clinical efficacy and safety of lenalidomide with dexamethasone (LD) in the treatment of relapse/refractory lightchain amyloidosis.
Methodology:Ten patients diagnosed relapse/refractory lightchain amyloidosis were enrolled in this retrospective study. Their clinical data were reviewed, the hematological and organ responses, treatment related adverse events were analyzed.
Results:Seven males and three females were enrolled with a median age of 645(568,688)years old. Of seven evaluable patients, overall hematological response rate was 429%, including 143% with complete remission. Heart response was seen in two patients,  none had renal response. All were alive and the overall survival and median progression free survival were not reached. Grade 3/4 adverse reaction rate was 30%.
Conclusion:Lenalidomide with dexamethasone was effective and welltolerated as a salvage treatment for relapse/refractory lightchain amyloidosis.

Key words: light-chain amyloidosis, lenalidomide, efficacy, adverse event