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了解高危冒烟型多发性骨髓瘤。

Understanding high-risk smoldering multiple myeloma.

作者信息

Kreiniz Natalia, Gertz Morie A

机构信息

Division of Hematology, Bnai Zion Medical Centre, Haifa, Israel.

The Ruth and Bruce Rappaport, Technion, Haifa, Israel.

出版信息

Leuk Lymphoma. 2023 Jul-Aug;64(8):1361-1372. doi: 10.1080/10428194.2023.2216818. Epub 2023 May 25.

Abstract

Smoldering multiple myeloma (SMM) is an asymptomatic condition with heterogeneous biology and various risks of progression to symptomatic disease. The best-known risk stratification models are Mayo-2018, and IWWG based on tumor burden. Recently, the personalized risk assessment tool PANGEA was introduced. New markers of SMM progression, including genomic and immune characteristics of plasma cells (PCs) and tumor microenvironment, are under investigation, and some have been incorporated into traditional scoring systems. Only one phase 3 clinical trial demonstrated an overall survival benefit of lenalidomide for high-risk SMM patients. The study has limitations, and most guidelines recommend observation or participation in clinical trials for high-risk SMM. High-intensity time-limited treatment strategies for high-risk SMM demonstrated deep responses in single-arm studies. But these treatments can cause adverse effects in asymptomatic patients.This review aims to understand better the risk of SMM progression from a clinical and biological point of view.

摘要

冒烟型多发性骨髓瘤(SMM)是一种无症状疾病,具有异质性生物学特性和进展为症状性疾病的多种风险。最著名的风险分层模型是基于肿瘤负荷的Mayo-2018模型和国际骨髓瘤工作组(IWWG)模型。最近,引入了个性化风险评估工具PANGEA。SMM进展的新标志物,包括浆细胞(PC)的基因组和免疫特征以及肿瘤微环境,正在研究中,一些已被纳入传统评分系统。只有一项3期临床试验证明来那度胺对高危SMM患者有总生存获益。该研究有局限性,大多数指南建议对高危SMM患者进行观察或参与临床试验。针对高危SMM的高强度限时治疗策略在单臂研究中显示出深度缓解。但这些治疗可能会给无症状患者带来不良反应。本综述旨在从临床和生物学角度更好地了解SMM进展的风险。

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