Thrombosis-Driven Disease Progression in JAK2-Mutant Polycythemia Vera and Essential Thrombocythemia: Reassessing Risk-Based Management

IF 10.1 1区 医学 Q1 HEMATOLOGY
Tiziano Barbui, Valerio De Stefano, Elena Rossi, Arianna Ghirardi, Alessandra Carobbio, Giuseppe Gaetano Loscocco, Annalisa Condorelli, Paola Guglielmelli
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引用次数: 0

Abstract

This paper explores emerging therapies in polycythemia vera and essential thrombocythemia, focusing on thrombosis as a driver of disease progression leading to myelofibrosis, blast phase, second cancers, and mortality. While the thrombosis rate in high-risk patients has declined, it remains persistently high in low-risk individuals, with most events being arterial. Inflammation driven by JAK2 V617F mutation plays a primary role in pathogenesis, and mounting evidence suggests arterial thrombosis itself can fuel a self-sustaining cycle of inflammation, thereby accelerating hematologic and systemic complications. Early intervention with cytoreductive and anti-inflammatory drugs may not only prevent incidental thrombosis but also disrupt this inflammatory circuit.

血小板形成驱动的疾病进展在jak2突变型真性红细胞增多症和原发性血小板增多症:重新评估基于风险的管理
本文探讨真性红细胞增多症和原发性血小板增多症的新疗法,重点关注血栓形成作为导致骨髓纤维化、母细胞期、第二次癌症和死亡率的疾病进展的驱动因素。虽然高危患者的血栓形成率已经下降,但在低危人群中,血栓形成率仍然居高不下,大多数是动脉血栓形成。由JAK2 V617F突变驱动的炎症在发病机制中起主要作用,越来越多的证据表明动脉血栓形成本身可以促进炎症的自我维持循环,从而加速血液学和全身并发症。早期干预使用细胞减肥药和抗炎药不仅可以预防偶发血栓形成,而且可以破坏这种炎症回路。
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来源期刊
CiteScore
15.70
自引率
3.90%
发文量
363
审稿时长
3-6 weeks
期刊介绍: The American Journal of Hematology offers extensive coverage of experimental and clinical aspects of blood diseases in humans and animal models. The journal publishes original contributions in both non-malignant and malignant hematological diseases, encompassing clinical and basic studies in areas such as hemostasis, thrombosis, immunology, blood banking, and stem cell biology. Clinical translational reports highlighting innovative therapeutic approaches for the diagnosis and treatment of hematological diseases are actively encouraged.The American Journal of Hematology features regular original laboratory and clinical research articles, brief research reports, critical reviews, images in hematology, as well as letters and correspondence.
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