硼替佐米联合地塞米松治疗原发性系统性淀粉样变性的疗效及安全性

[The effects and safety of bortezomib combined with dexamethasone in the treatment of primary systemic amyloidosis].

作者信息

Li Juan, Zhao Ying, Zeng Li-jin, Su Chang, Wang He-hua

机构信息

Department of Hematology, the First Affiliated Hospital of Sun Yat-sen University, Guangzhou 510080, China.

出版信息

Zhonghua Nei Ke Za Zhi. 2009 Sep;48(9):741-4.

PMID:20079210

Abstract

OBJECTIVE

To evaluate the effects and safety of the regimen of bortezomib combined with dexamethasone (VD) in the treatment of primary systemic (AL) amyloidosis.

METHODS

Five newly diagnosed AL amyloidosis patients confirmed by renal biopsy with a median of 3 organs involved (3 to 5 organs) were treated by VD regimen for 3 (1-4) cycles.

RESULTS

Among 3 evaluable patients, 1 was in stable condition and 2 had hematologic response (partial remission and complete remission) and organ function improvement. Hematologic responses were rapid (median 1.5 cycles) and median time to organ response was 2 cycles. Three cases were survived and the periods of follow up were 5, 4 and 4 months respectively. The other 2 died 2 and 14 months after diagnosis. The side effects were asthenia, diarrhea, constipation, edema aggravation and fever, all of which were in I grade. No treatment associating death was found.

CONCLUSION

VD regimen might be an efficient, rapid effective and safe regimen in the treatment of AL amyloidosis.

摘要

目的

评估硼替佐米联合地塞米松（VD）方案治疗原发性系统性（AL）淀粉样变性的疗效及安全性。

方法

5例经肾活检确诊的初治AL淀粉样变性患者，累及器官中位数为3个（3至5个器官），接受VD方案治疗3（1至4）个周期。

结果

3例可评估患者中，1例病情稳定，2例有血液学反应（部分缓解和完全缓解）且器官功能改善。血液学反应迅速（中位数1.5个周期），器官反应的中位时间为2个周期。3例存活，随访时间分别为5、4和4个月。另外2例在诊断后2个月和14个月死亡。副作用为乏力、腹泻、便秘、水肿加重和发热，均为I级。未发现与治疗相关的死亡。