Purpose of Review

Philadelphia-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), prefibrotic (pre-), and overt-primary myelofibrosis (primary MF, PMF). PV and ET could evolve into secondary MF (SMF), whose early diagnosis relies on monitoring signs of possible progression. All MPNs have a risk of blast phase (BP), that is associated with a very dismal outcome. Overall survival (OS) is different among MPNs, and disease-specific prognostic scores should be applied for a correct clinical management. In this review, an overview of current prognostic scores in MPNs will be provided.

Recent Findings

The biological complexity of MPNs and its role on the trajectory of disease outcome have led to the design of integrated prognostic models that are nowadays of common use in PMF patients. As for PV and ET, splicing gene mutations could have a detrimental role, but with the limit of the not routinary recommended application of extensive molecular analysis in these diseases. SMF is recognized as a distinct entity compared to PMF, and OS estimates should be calculated by the MYSEC-PM (Myelofibrosis SECondary-prognostic model). Both in PMF and SMF, decisions as selection of patients potentially candidates to allogenic stem cell transplant or that could benefit from an early shift from standard treatment are based not only on conventional prognostic scores, but also on multivariable algorithms.

Summary

The expanding landscape of risk prediction for OS, evolution to BP, and SMF progression from PV/ET informs personalized approach to the management of patients affected by MPNs.

中文翻译：

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费城阴性骨髓增生性肿瘤预后的个性化定义

审查目的

费城阴性骨髓增生性肿瘤 (MPN) 包括真性红细胞增多症 (PV)、原发性血小板增多症 (ET)、纤维化前 (pre-) 和明显原发性骨髓纤维化 (primary MF, PMF)。PV 和 ET 可能演变为继发性 MF (SMF)，其早期诊断依赖于监测可能进展的迹象。所有 MPN 都有爆发期 (BP) 的风险，这与非常令人沮丧的结果相关。MPN 的总生存期 (OS) 不同，应应用疾病特异性预后评分进行正确的临床管理。在本次审查中，将提供 MPN 中当前预后评分的概述。

最近的发现

MPN 的生物学复杂性及其在疾病结果轨迹中的作用导致设计了目前在 PMF 患者中普遍使用的综合预后模型。至于 PV 和 ET，剪接基因突变可能会产生有害作用，但受限于在这些疾病中广泛分子分析的非常规推荐应用。与 PMF 相比，SMF 被认为是一个不同的实体，并且 OS 估计值应通过 MYSEC-PM（骨髓纤维化二级预后模型）计算。在 PMF 和 SMF 中，选择可能接受同种异体干细胞移植或可能受益于标准治疗早期转变的患者的决定不仅基于传统的预后评分，还基于多变量算法。

概括

OS 风险预测的扩展前景、向 BP 的演变以及从 PV/ET 的 SMF 进展为管理受 MPN 影响的患者提供了个性化方法。