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病例报告:轻链淀粉样变性对塞利尼索联合达雷妥尤单抗及地塞米松(SDd)治疗有反应。

Case report: Light-chain amyloidosis responsive to selinexor in combination with daratumumab and dexamethasone (SDd) therapy.

作者信息

Long Xiaolu, An Ning, Li Chunhui, Zhu Hui, Li Haojie, Yu Qiuxia, Que Yimei, Xu Menglei, Li Zhe, Chen Wei, Wang Shuai, Wang Di, Li Chunrui

机构信息

Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.

Immunotherapy Research Center for Hematologic Diseases of Hubei Province, Wuhan, Hubei, China.

出版信息

Front Med (Lausanne). 2024 May 2;11:1363805. doi: 10.3389/fmed.2024.1363805. eCollection 2024.

Abstract

The outcome of AL amyloidosis remains poor, particularly in patients with advanced organ involvement which takes long time to recovery. We conducted an observational study of two patients with AL amyloidosis treated with SDd regimen. Both patients successfully achieved significant hematological and organ responses without severe adverse events, and the time to organ response was remarkably shorter than previously reported. Notably, an over 15% reduction in interventricular septal thickness (IVST) was observed in patient#2 within 6 months. Up to now, SDd therapy has not been previously reported in AL amyloidosis and may be a promising option for these patients.

摘要

AL型淀粉样变性的预后仍然很差,尤其是在器官受累严重且恢复时间长的患者中。我们对两名接受SDd方案治疗的AL型淀粉样变性患者进行了一项观察性研究。两名患者均成功实现了显著的血液学和器官反应,且无严重不良事件,器官反应时间明显短于先前报道。值得注意的是,患者2在6个月内室间隔厚度(IVST)减少了超过15%。到目前为止,SDd疗法在AL型淀粉样变性中尚未见报道,可能是这些患者的一个有前景的选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8dd5/11096580/50310fd887ef/fmed-11-1363805-g0001.jpg

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