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轻链沉积病的治疗:一项系统综述

Treatment of Light Chain Deposition Disease: A Systematic Review.

作者信息

Masood Adeel, Ehsan Hamid, Iqbal Qamar, Salman Ahmed, Hashmi Hamza

机构信息

Hematology/Oncology, MD Anderson Cancer Center, Houston, TX 77030, USA.

Hematology/Oncology, Levine Cancer Institute/Atrium Health, Charlotte, NC, USA.

出版信息

J Hematol. 2022 Aug;11(4):123-130. doi: 10.14740/jh1038. Epub 2022 Aug 30.

Abstract

Light chain deposition disease (LCDD) is a rare hematologic disorder that can affect any organ but predominantly involves the kidneys. Existing literature is limited to case reports and small single-center retrospective series, explaining the lack of any treatment algorithms and management guidelines for patients with this disorder. In this systematic review of literature, we explored the role of standard and high-dose chemotherapy-autologous stem cell transplant for LCDD. A total of 11 studies were identified to evaluate the hematologic and renal responses to various treatment regimens. Autologous stem cell transplant and bortezomib-based regimens appear to have reasonable safety and efficacy for this rare hematologic disorder, albeit some statistical and analytical limitations. Large multicenter retrospective and prospective studies are needed to better elucidate the role of various chemotherapy regimens as well as autologous stem cell transplant for patients with LCDD.

摘要

轻链沉积病(LCDD)是一种罕见的血液系统疾病,可累及任何器官,但主要累及肾脏。现有文献仅限于病例报告和小型单中心回顾性系列研究,这解释了为何缺乏针对该疾病患者的治疗算法和管理指南。在这项系统性文献综述中,我们探讨了标准和大剂量化疗联合自体干细胞移植在LCDD治疗中的作用。共纳入11项研究,以评估各种治疗方案的血液学和肾脏反应。自体干细胞移植和基于硼替佐米的治疗方案对于这种罕见的血液系统疾病似乎具有合理的安全性和疗效,尽管存在一些统计和分析上的局限性。需要开展大型多中心回顾性和前瞻性研究,以更好地阐明各种化疗方案以及自体干细胞移植在LCDD患者治疗中的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8bbc/9451548/3ebd966cff87/jh-11-123-g001.jpg

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