BCR::ABL1-negative myeloproliferative neoplasms (MPN) are clonal hematological malignancies resulting from the proliferation of myeloid cells harboring a JAK-STAT pathway activating driver mutation. MPN management recommendations are based on the evaluation of different risks to prevent disease evolution associated events while preserving patients' quality of life. Such risks can be common across all MPN or specific to each subtype (polycythemia vera (PV), essential thrombocythemia (ET), prefibrotic myelofibrosis (pre-MF), primary myelofibrosis (PMF)). MF-patients harbor the worse prognosis and hematopoietic stem cell transplantation (HSCT) is the only curative treatment, at the expense of a high morbi-mortality. Therefore, accurate scoring systems to estimate overall survival are crucial for MF patients' management and selection for HSCT. In PV and ET, vascular events prediction is prioritized given their higher incidence and related morbi-mortality. Finally, quality of life evaluation is important for all subtypes. To predict these risks and adapt MPN therapeutic strategies, clinical risk scores have been developed over the past decades, more recently including molecular risk factors for more accurate risk stratification. The large number of scoring systems available in combination with disease heterogeneity and the necessity to predict diverse outcomes, make it difficult for clinicians to choose the most appropriate score to evaluate their patients' risk in 2024. Here, we provide an overview of MPN disease evolution associated events incidence and conduct an exhaustive comparative review of the scoring systems currently available for each risk. Finally, we propose an algorithm for the use of these scores in clinical practice in each MPN subtype.

中文翻译：

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当前用于临床实践的骨髓增生性肿瘤评分系统。


BCR：：ABL1 阴性骨髓增生性肿瘤 （MPN） 是由携带 JAK-STAT 通路激活驱动突变的髓系细胞增殖引起的克隆性血液系统恶性肿瘤。MPN 管理建议基于对不同风险的评估，以防止疾病演变相关事件，同时保持患者的生活质量。此类风险在所有 MPN 中可能常见，也可能特定于每种亚型 （真性红细胞增多症 （PV）、原发性血小板增多症 （ET）、纤维化前骨髓纤维化 （pre-MF）、原发性骨髓纤维化 （PMF））。MF 患者的预后较差，造血干细胞移植 （HSCT） 是唯一的治愈性治疗方法，代价是死亡率高。因此，用于估计总生存期的准确评分系统对于 MF 患者的管理和 HSCT 选择至关重要。在 PV 和 ET 中，鉴于血管事件的发病率和相关的死亡率较高，因此优先考虑血管事件预测。最后，生活质量评估对所有亚型都很重要。为了预测这些风险并调整 MPN 治疗策略，过去几十年已经开发了临床风险评分，最近包括分子风险因素，以便更准确地进行风险分层。大量可用的评分系统与疾病异质性以及预测不同结果的必要性相结合，使临床医生难以选择最合适的评分来评估 2024 年患者的风险。在这里，我们概述了 MPN 疾病演变相关事件的发生率，并对目前可用于每种风险的评分系统进行了详尽的比较审查。最后，我们提出了一种算法，用于在临床实践中为每个 MPN 亚型使用这些分数。