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环磷酰胺、硼替佐米和甲泼尼龙(CyBorMe)治疗AL淀粉样变性:单中心初步经验

Cyclophosphamide, Bortezomib and Methylprednisolone (CyBorMe) for the Treatment of AL Amyloidosis: Initial Experience From a Single Center.

作者信息

Jimenez-Zepeda Victor H, Lee Holly, Fine Nowell, McCulloch Sylvia, Tay Jason, Duggan Peter, Neri Paola, Bahlis Nizar

机构信息

Tom Baker Cancer Center/University of Calgary, 1331 29th St, NW, Calgary, AB T2N 4N2 Canada.

Division of Cardiology, Department of Cardiac Sciences, Libin Cardiovascular Institute of Alberta, Calgary, AB Canada.

出版信息

Indian J Hematol Blood Transfus. 2021 Oct;37(4):675-678. doi: 10.1007/s12288-021-01406-z. Epub 2021 Feb 17.

Abstract

The use of cyclophosphamide, bortezomib and dexamethasone (CyBorD) is widely accepted in the treatment of AL amyloidosis (AL). Recently, the substitution of dexamethasone by methylprednisolone (CyBorMe) appeared to improve response rates and survival outcomes. All consecutive newly diagnosed AL amyloidosis treated with CyBorMe from 01/19 to 08/20 were evaluated. A historic cohort of patients treated with CyBorD was used for comparison (01/13-08/20). Methylprednisolone was given IV at 500 mg weekly for 4 weeks in the CyBorMe group. 43 patients were treated with CyBorD and 14 with CyBorMe. After a median of 4 cycles of CyBorD and 3 cycles of CyBorMe, Hematological Response was seen in 90.6% and 92.8% of cases, including CR in 28.5% and 35.7%, VGPR in 33.3% and 35.7% and PR in 30.9% and 21.4% for CyBorD and CyBorMe, respectively. Time to first response was faster in the CyBorMe group (4 vs. 6 weeks) and cardiac response was observed in 44% and 31% of patients treated with CyBorMe and CyBorD, respectively. CyBorMe appeared to be efficacious and well tolerated in patients with AL amyloidosis. Prospective studies with CyBorMe in the stage III/IV group are warranted aiming to minimize toxicity.

摘要

环磷酰胺、硼替佐米和地塞米松(CyBorD)联合使用在治疗轻链型淀粉样变性(AL)中已被广泛接受。最近,用地塞米松替代为甲泼尼龙(CyBorMe)似乎能提高缓解率和生存结果。对2019年1月至2020年8月期间所有接受CyBorMe治疗的新诊断AL淀粉样变性患者进行了评估。使用一组接受CyBorD治疗的历史队列患者作为对照(2013年1月至2020年8月)。在CyBorMe组中,甲泼尼龙静脉注射,每周500mg,共4周。43例患者接受了CyBorD治疗,14例接受了CyBorMe治疗。CyBorD组和CyBorMe组分别经过中位数4个周期和3个周期治疗后,血液学缓解率分别为90.6%和92.8%,其中完全缓解(CR)分别为28.5%和35.7%,非常好的部分缓解(VGPR)分别为33.3%和35.7%,部分缓解(PR)分别为30.9%和21.4%。CyBorMe组首次缓解时间更快(4周对6周),接受CyBorMe和CyBorD治疗的患者心脏缓解率分别为44%和31%。CyBorMe在AL淀粉样变性患者中似乎有效且耐受性良好。有必要对III/IV期组患者进行CyBorMe的前瞻性研究,以尽量减少毒性。

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