How low risk are low risk myelodysplastic syndromes?
How low risk are low risk myelodysplastic syndromes?
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DOI：
10.1080/17474086.2022.2029698
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发表时间：
2022-01
影响因子：
2.8
通讯作者：
A. DeZern;W. Dalton
中科院分区：
文献类型：
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作者：
A. DeZern;W. Dalton
摘要
引言
风险分层对于许多疾病的恰当管理至关重要，但在骨髓增生异常综合征（MDS）患者中，其预期生存期可能差异很大，准确的疾病预后尤其重要。MDS相对缺乏治疗方法这一点进一步支持了这一观点，因此我们必须谨慎且准确地进行预后判断。目前，使用临床预后评分系统，MDS患者通常被分为高风险（HR）和低风险（LR）疾病，但这些系统存在局限性。
涵盖领域
作者回顾了有关MDS的诊断、预后、治疗和结果的文献。国际预后评分系统修订版（IPSS - R）未涵盖的疾病病因、特定临床特征或分子遗传学信息等因素可能改变风险分层，并识别出一部分实际上表现更像高风险MDS的低风险MDS患者。
专家意见
本综述将描述目前对那些病情可能比IPSS - R预测的更不利的低风险MDS患者的识别和管理。作者对被认为会使患者从低风险疾病升级为高风险疾病的临床和分子特征进行了评论。
ABSTRACT Introduction 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. Areas covered 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. Expert opinion 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.