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将突变和骨髓纤维化纳入修订后的骨髓增生异常综合征国际预后评分系统。

Incorporating mutations and bone marrow fibrosis into the revised international prognostic scoring system in myelodysplastic syndromes.

机构信息

Department of Hematology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.

出版信息

Leuk Lymphoma. 2024 Jan;65(1):100-108. doi: 10.1080/10428194.2023.2271593. Epub 2024 Jan 10.

Abstract

The independent prognostic significance of bone marrow fibrosis (BMF) in myelodysplastic syndromes (MDS) is challenged under currently molecular prognostic models. In this study, the clinical and genetic data from 438 MDS patients were analyzed retrospectively. The patients were randomly divided into training ( = 306) and validation ( = 132) cohorts. The independent significant prognostic factors included age, IPSS-R, BMF, TP53 and U2AF1. Using their weighted coefficients, we developed a simplified prognostic system. Four risk groups were produced: low, intermediate, high and very high. The new model yielded more clearly separated survival curves than the IPSS-R. In addition, our model achieved higher C-indexes (0.61 in the training cohort and 0.63 in the validation cohort) than the IPSS-RM model (0.59 and 0.58) and IPSS-R (0.57 and 0.56). In conclusion, BMF was an independent significant prognostic factor for MDS, and adding BMF into the IPSS-R improved its predictive capability.

摘要

骨髓纤维化(BMF)在骨髓增生异常综合征(MDS)中的独立预后意义在目前的分子预后模型下受到挑战。本研究回顾性分析了 438 例 MDS 患者的临床和基因数据。患者被随机分为训练(n=306)和验证(n=132)队列。独立的显著预后因素包括年龄、IPSS-R、BMF、TP53 和 U2AF1。使用它们的加权系数,我们开发了一个简化的预后系统。产生了四个风险组:低、中、高和非常高。新模型产生的生存曲线比 IPSS-R 更清晰地分开。此外,我们的模型在训练队列和验证队列中的 C 指数(分别为 0.61 和 0.63)均高于 IPSS-RM 模型(分别为 0.59 和 0.58)和 IPSS-R(分别为 0.57 和 0.56)。总之,BMF 是 MDS 的一个独立的显著预后因素,将 BMF 加入 IPSS-R 可提高其预测能力。

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