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.
Thrombosis‐Driven Disease Progression in JAK2‐Mutant Polycythemia Vera and Essential Thrombocythemia: Reassessing Risk‐Based Management / Barbui, Tiziano; Stefano, Valerio De; Rossi, Elena; Ghirardi, Arianna; Carobbio, Alessandra; Loscocco, Giuseppe Gaetano; Condorelli, Annalisa; Guglielmelli, Paola. - In: AMERICAN JOURNAL OF HEMATOLOGY. - ISSN 0361-8609. - ELETTRONICO. - 100:(2025), pp. 66-73. [10.1002/ajh.27657]
Thrombosis‐Driven Disease Progression in JAK2‐Mutant Polycythemia Vera and Essential Thrombocythemia: Reassessing Risk‐Based Management
Loscocco, Giuseppe Gaetano;Guglielmelli, Paola
2025
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.| File | Dimensione | Formato | |
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