Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Francesco Sforza, 35 - 20122, Milan, Italy.
Dipartimento Di Oncologia Ed Onco-Ematologia, Università Degli Studi Di Milano, Via Francesco Sforza, 35 - 20122, Milan, Italy.
Curr Hematol Malig Rep. 2024 Oct;19(5):223-235. doi: 10.1007/s11899-024-00739-6. Epub 2024 Aug 24.
Myelofibrosis (MF) includes prefibrotic primary MF (pre-PMF), overt-PMF and secondary MF (SMF). Median overall survival (OS) of pre-PMF, overt-PMF and SMF patients is around 14 years, seven and nine years, respectively. Main causes of mortality are non-clonal progression and transformation into blast phase.
Discoveries on the impact of the biological architecture on OS have led to the design of integrated scores to predict survival in PMF. For SMF, OS estimates should be calculated by the specific MYSEC-PM (MYelofibrosis SECondary-prognostic model). Information on the prognostic role of the molecular landscape in SMF is accumulating. Crucial treatment decisions for MF patients could be now supported by multivariable predictive algorithms. OS should become a relevant endpoint of clinical trials. Prognostic models guide prediction of OS and treatment planning in MF, therefore, their timely application is critical in the personalized approach of MF patients.
骨髓纤维化（MF）包括纤维化前期原发性骨髓纤维化（pre-PMF）、显性骨髓纤维化（overt-PMF）和继发性骨髓纤维化（SMF）。pre-PMF、overt-PMF 和 SMF 患者的中位总生存期（OS）分别约为 14 年、7 年和 9 年。死亡的主要原因是非克隆进展和向 blast 期转化。
对生物学结构对 OS 的影响的研究发现，导致了设计综合评分来预测 PMF 的生存。对于 SMF，OS 估计值应该通过特定的 MYSEC-PM（骨髓纤维化继发性预后模型）来计算。关于 SMF 中分子图谱预后作用的信息正在积累。MF 患者的关键治疗决策现在可以得到多变量预测算法的支持。OS 应该成为临床试验的一个相关终点。预后模型指导 MF 中 OS 和治疗计划的预测，因此，在 MF 患者的个体化治疗中，及时应用它们至关重要。
