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Review
. 2025 Jun:100 Suppl 4:66-73.
doi: 10.1002/ajh.27657. Epub 2025 Mar 10.

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

Tiziano Barbui  1 , Valerio De Stefano  2   3 , Elena Rossi  2   3 , Arianna Ghirardi  1 , Alessandra Carobbio  4 , Giuseppe Gaetano Loscocco  5 , Annalisa Condorelli  6 , Paola Guglielmelli  5
Affiliations
Review

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

Tiziano Barbui et al. Am J Hematol. 2025 Jun.

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.

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References

    1. D. A. Arber, A. Orazi, R. P. Hasserjian, et al., “International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: Integrating Morphologic, Clinical, and Genomic Data,” Blood 140 (2022): 1200–1228.
    1. J. Thiele, H. M. Kvasnicka, A. Orazi, et al., “The International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: Myeloproliferative Neoplasms,” American Journal of Hematology 98 (2023): 166–179.
    1. A. M. Vannucchi and P. Guglielmelli, “Acute Myocardial Infarction and Chronic Myeloproliferative Neoplasms: Friend and Enemy, Depending on Circumstances,” Cardio Oncology 5 (2023): 469–471.
    1. A. Tefferi and T. Barbui, “Polycythemia Vera: 2024 Update on Diagnosis, Risk‐Stratification, and Management,” American Journal of Hematology 98 (2023): 1465–1487.
    1. A. Tefferi and T. Barbui, “Bcr/Abl‐Negative, Classic Myeloproliferative Disorders: Diagnosis and Treatment,” Mayo Clinic Proceedings 80 (2005): 1220–1232.

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