Division of Hematologic Malignancies, Sidney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Expert Rev Hematol. 2022 Jan;15(1):15-24. doi: 10.1080/17474086.2022.2029698. Epub 2022 Jan 24.
Risk stratification is crucial to the appropriate management of many diseases, but in patients with myelodysplastic syndromes (MDS), for whom expected survival can vary greatly, accurate disease prognostication is especially important. This is further supported by a relative lack of therapies in MDS, and thus we must prognosticate carefully and accurately. Currently, patients with MDS are often grouped into higher-risk (HR) versus lower-risk (LR) disease using clinical prognostic scoring systems, but these systems have limitations.
The authors reviewed the literature on diagnostics, prognostics, therapeutics and outcomes in MDS. Factors such as disease etiology, specific clinical characteristics, or molecular genetic information not captured in the international prognostic scoring system revised IPSS-R can alter risk stratification, and identify a subset of LR-MDS patients who actually behave more like HR-MDS.
This review will describe the current identification and management of patients with LR MDS disease whose condition is likely to behave in a less favorable manner than predicted by the IPSS-R. The authors comment on clinical and molecular features which are believe to upstage a patient from lower to higher risk disease.
风险分层对于许多疾病的适当管理至关重要，但对于骨髓增生异常综合征（MDS）患者，由于其预期生存时间差异很大，因此准确的疾病预后预测尤为重要。这进一步支持了 MDS 治疗方法相对较少，因此我们必须仔细、准确地进行预后预测。目前，MDS 患者通常使用临床预后评分系统分为高危（HR）与低危（LR）疾病，但这些系统存在局限性。
作者回顾了 MDS 的诊断、预后、治疗和结果的文献。疾病病因、特定临床特征或国际预后评分系统修订版（IPSS-R）中未捕获的分子遗传信息等因素可以改变风险分层，并确定一部分 LR-MDS 患者的实际表现更像 HR-MDS。
本综述将描述目前对 LR-MDS 疾病患者的识别和管理，这些患者的病情表现可能不如 IPSS-R 预测的那样有利。作者评论了一些临床和分子特征，这些特征被认为可使患者从低危疾病升级为高危疾病。
