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Are MPNs Vascular Diseases?

  • Myeloproliferative Disorders (JJ Kiladjian, Section Editor)
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Abstract

A high risk of arterial and venous thrombosis is the hallmark of chronic myeloproliferative neoplasms (MPNs), particularly polycythemia vera (PV) and essential thrombocythemia (ET). Clinical aspects, pathogenesis and management of thrombosis in MPN resemble those of other paradigmatic vascular diseases. The occurrence of venous thrombosis in atypical sites, such as the splanchnic district, and the involvement of plasmatic prothrombotic factors, including an acquired resistance to activated protein C, both link MPN to inherited thrombophilia. Anticoagulants are the drugs of choice for these complications. The pathogenic role of leukocytes and inflammation, and the high mortality rate from arterial occlusions are common features of MPN and atherosclerosis. The efficacy and safety of aspirin in reducing deaths and major thrombosis in PV have been demonstrated in a randomized clinical trial. Finally, the Virchow’s triad of impaired blood cells, endothelium and blood flow is shared both by MPN and thrombosis in solid cancer. Phlebotomy and myelosuppressive agents are the current therapeutic options for correcting these abnormalities and reducing thrombosis in this special vascular disease represented by MPN.

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Acknowledgments

Dr. Barbui and Dr. Finazzi are supported by a grant from Associazione Italiana per la Ricerca sul Cancro (AIRC, Milano) “Special Program Molecular Clinical Oncology 5x1000” to AGIMM (AIRC287 Gruppo Italiano Malattie Mieloproliferative) (project #1005).

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Conflict of Interest

Guido Finazzi declares that he has no conflict of interest.

Valerio De Stefano has received research support from Shire, and payment for the development of educational presentations including service on speakers bureaus from Shire and Novartis.

Tiziano Barbui has received honoraria from Novartis for serving on an advisory board.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Authors and Affiliations

  1. Division of Hematology, Ospedale Papa Giovanni XXIII, Piazza OMS, 1, 24127, Bergamo, BG, Italy

    Guido Finazzi & Tiziano Barbui

  2. Research Foundation, Hospital Papa Giovanni XXIII, Bergamo, Italy

    Tiziano Barbui

  3. Institute of Hematology, Department of Medical Sciences, Catholic University, Rome, Italy

    Valerio De Stefano

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  1. Guido Finazzi
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Correspondence to Guido Finazzi.

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Finazzi, G., De Stefano, V. & Barbui, T. Are MPNs Vascular Diseases?. Curr Hematol Malig Rep 8, 307–316 (2013). https://doi.org/10.1007/s11899-013-0176-z

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