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Therapy of Polycythemia Vera and Essential Thrombocythemia

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Myeloproliferative Neoplasms

Part of the book series: Contemporary Hematology ((CH))

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Abstract

Major causes of morbidity and mortality in polycythemia vera (PV) and essential thrombocythemia (ET) are represented by thrombosis and bleeding, progression to myelofibrosis, and transformation to acute leukemia. Myelosuppressive therapy, preferentially with hydroxyurea, can reduce the rate of vascular complications, but there is some concern about an increased rate of leukemic transformation with this agent. Therefore, management of these disorders poses a significant challenge, and a risk-oriented therapeutic approach should be followed to avoid inappropriate exposure to cytotoxic drugs on one side or suboptimal treatment on the other. Established risk factors for cardiovascular events are represented by older age and previous thrombosis, while impact of novel biological factors, including leukocytosis and JAK2V617F mutational status and/or mutational burden, is under investigation. Low-risk PV patients should be managed only with phlebotomy and aspirin, while high-risk patients should also receive cytotoxic therapy. Regarding the management of ET, there is no clear indication for intervention in low-risk patients, while high-risk patients should be managed with chemotherapy. Other therapeutic options, such as interferon alpha or anagrelide, may find place in selected patients including those who are resistant/intolerant to hydroxyurea.

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Author information

Authors and Affiliations

  1. Department of Hematology, Ospedali Riuniti di Bergamo, Bergamo, Italy

    Guido Finazzi

  2. Research Foundation, Ospedali Riuniti di Bergamo, Bergamo, Italy

    Tiziano Barbui

Authors
  1. Guido Finazzi
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  2. Tiziano Barbui
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Correspondence to Guido Finazzi .

Editor information

Editors and Affiliations

  1. M.D. Anderson Cancer Center, Dept. Leukemia, University of Texas, Holcombe Blvd. 1515, Houston, 77030, Texas, USA

    Srdan Verstovsek

  2. Dept. Internal Medicine, Div. Hematology, Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    Ayalew Tefferi

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Finazzi, G., Barbui, T. (2011). Therapy of Polycythemia Vera and Essential Thrombocythemia. In: Verstovsek, S., Tefferi, A. (eds) Myeloproliferative Neoplasms. Contemporary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-266-7_5

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  • DOI: https://doi.org/10.1007/978-1-60761-266-7_5

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  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-60761-265-0

  • Online ISBN: 978-1-60761-266-7

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