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中华肾病研究电子杂志 ›› 2014, Vol. 03 ›› Issue (02) : 60 -64. doi: 10.3877/cma.j.issn.2095-3216.2014.02.001

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重视AL 型肾淀粉样变的规范治疗
章友康,1, 姚英2   
  1. 1.100034 北京大学第一医院肾内科
    2.首都医科大学附属复兴医院肾内科
  • 出版日期:2014-04-15
  • 通信作者: 章友康

Attention to standard treatment of renal immunoglobulin light-chain amyloidosis

Youkang Zhang,1, Ying Yao1   

  1. 1.Renal Division, Peking University First Hospital, Beijing 100034, China
  • Published:2014-04-15
  • Corresponding author: Youkang Zhang
引用本文:

章友康, 姚英. 重视AL 型肾淀粉样变的规范治疗[J/OL]. 中华肾病研究电子杂志, 2014, 03(02): 60-64.

Youkang Zhang, Ying Yao. Attention to standard treatment of renal immunoglobulin light-chain amyloidosis[J/OL]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2014, 03(02): 60-64.

我国肾脏淀粉样变患者最常见的类型是免疫球蛋白轻链(AL)型淀粉样变,占绝大多数。 未经治疗的AL 型肾淀粉样变患者预后很差,中位生存时间只有6 ~12 个月。 近年来,随着早期诊断及治疗的进展,AL 型肾淀粉样变患者的预后有明显改善。 AL 肾淀粉样变的治疗经历了三个重要的阶段。 最初的标准化疗方案是美法仑联合强的松,但疗效不尽人意;大剂量美法仑联合自体干细胞移植治疗,使得治疗组患者的生存时间明显延长,但是仅有不到四分之一的患者适合该疗法;近年来美法仑联合地塞米松同样可使治疗组患者生存期明显延长,被列为治疗AL 型肾淀粉样变的一线方案。 另外,硼替佐米、沙利度胺等一些新型制剂的应用,也为AL 型肾淀粉样变的治疗提供了更多的选择。

Immunoglobulin light-chain (AL) amyloidosis is the most common type of renal amyloidosis in China. Untreated patients of this disease have a dismal outcome, with a median survival of 6-12 months from diagnosis. In recent years, with the progress of early diagnosis and treatment, the prognosis of patients with AL-type renal amyloidosis have been improved significantly. The treatment of AL-type renal amyloidosis has gone through three major phases. The initial standard chemotherapy is melphalan plus prednisone, but the effect was unsatisfactory. High-dose melphalan plus autologous stem cell transplantation substantially prolonged the survival time of patients, but only less than a quarter of the patients were suitable for the therapy. Currently, melphalan plus dexamethasone also significantly prolonged the survival of patients, and has been is listed as the first-line treatment of AL-type renal amyloidosis. In addition, some new preparations, such bortezomib and thalidomide, etc, have offered more choices for the treatment of AL-type renal amyloidosis.

表1 AL 型肾淀粉样变治疗后血液和器官疗效反应的判定标准[11]
图1 免疫球蛋白轻链型淀粉样变治疗指南(一) 注:AL 为免疫球蛋白轻链,HDM-ASCT 为大剂量美法仑联合自体干细胞移植,dFLC 为血清中两种游离轻链的差值,a Mayo 分期Ⅲ期(心脏同种型肌钙蛋白T >0.035 μg/L 和脑钠肽的N 末端前体肽>332 ng/L),b如果器官受累发生进展,随时采用其他治疗方案
图2 免疫球蛋白轻链型淀粉样变治疗指南(二) 注:AL 为免疫球蛋白轻链,dFLC 为血清中两种游离轻链的差值,a Mayo 分期Ⅲ期(心脏同种型肌钙蛋白T >0.035 μg/L 和脑钠肽的N 末端前体肽>332 ng/L), b 如果器官受累发生进展,随时采用其他治疗方案
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