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Polycythemia Vera (PV): Update on Emerging Treatment Options

Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by exuberant red cell production leading to a broad range of symptoms that compromise quality of life and productivity of patients. PV reduces survival expectation, primarily due to thrombotic events, transformation to bla...

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Autores principales: Benevolo, Giulia, Vassallo, Francesco, Urbino, Irene, Giai, Valentina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7981161/
https://www.ncbi.nlm.nih.gov/pubmed/33758507
http://dx.doi.org/10.2147/TCRM.S213020
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author Benevolo, Giulia
Vassallo, Francesco
Urbino, Irene
Giai, Valentina
author_facet Benevolo, Giulia
Vassallo, Francesco
Urbino, Irene
Giai, Valentina
author_sort Benevolo, Giulia
collection PubMed
description Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by exuberant red cell production leading to a broad range of symptoms that compromise quality of life and productivity of patients. PV reduces survival expectation, primarily due to thrombotic events, transformation to blast phase and post-PV myelofibrosis or to development of second cancers, which are associates with poor prognosis. Current therapeutic first line recommendations based on risk adapted classification divided patients into two groups, according to age (< or >60 years) and presence of prior thrombotic events. Low-risk patients (age <60 years and no prior history of thrombosis) should be treated with aspirin (81–100 mg/d) and phlebotomy, to maintain hematocrit <45%. High-risk patients (age >60 years and/or prior history of thrombosis), in addition to aspirin and phlebotomies, should receive cytoreductive therapy in order to reduce thrombotic risk. Nowadays hydroxyurea still remains the cytoreductive agent of first choice, reserving Interferon to young patients or childbearing women. During the last years, ruxolitinib emerged as a new treatment in PV patients, as second line therapy: it appeared especially effective in patients with severe pruritus, symptomatic splenomegaly, or post-PV myelofibrosis symptoms. Currently, in PV treatment, several molecules have been tested or are under investigation. At present, the drug that has shown the most encouraging results is givinostat.
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spelling pubmed-79811612021-03-22 Polycythemia Vera (PV): Update on Emerging Treatment Options Benevolo, Giulia Vassallo, Francesco Urbino, Irene Giai, Valentina Ther Clin Risk Manag Review Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by exuberant red cell production leading to a broad range of symptoms that compromise quality of life and productivity of patients. PV reduces survival expectation, primarily due to thrombotic events, transformation to blast phase and post-PV myelofibrosis or to development of second cancers, which are associates with poor prognosis. Current therapeutic first line recommendations based on risk adapted classification divided patients into two groups, according to age (< or >60 years) and presence of prior thrombotic events. Low-risk patients (age <60 years and no prior history of thrombosis) should be treated with aspirin (81–100 mg/d) and phlebotomy, to maintain hematocrit <45%. High-risk patients (age >60 years and/or prior history of thrombosis), in addition to aspirin and phlebotomies, should receive cytoreductive therapy in order to reduce thrombotic risk. Nowadays hydroxyurea still remains the cytoreductive agent of first choice, reserving Interferon to young patients or childbearing women. During the last years, ruxolitinib emerged as a new treatment in PV patients, as second line therapy: it appeared especially effective in patients with severe pruritus, symptomatic splenomegaly, or post-PV myelofibrosis symptoms. Currently, in PV treatment, several molecules have been tested or are under investigation. At present, the drug that has shown the most encouraging results is givinostat. Dove 2021-03-16 /pmc/articles/PMC7981161/ /pubmed/33758507 http://dx.doi.org/10.2147/TCRM.S213020 Text en © 2021 Benevolo et al. http://creativecommons.org/licenses/by-nc/3.0/ This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php).
spellingShingle Review
Benevolo, Giulia
Vassallo, Francesco
Urbino, Irene
Giai, Valentina
Polycythemia Vera (PV): Update on Emerging Treatment Options
title Polycythemia Vera (PV): Update on Emerging Treatment Options
title_full Polycythemia Vera (PV): Update on Emerging Treatment Options
title_fullStr Polycythemia Vera (PV): Update on Emerging Treatment Options
title_full_unstemmed Polycythemia Vera (PV): Update on Emerging Treatment Options
title_short Polycythemia Vera (PV): Update on Emerging Treatment Options
title_sort polycythemia vera (pv): update on emerging treatment options
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7981161/
https://www.ncbi.nlm.nih.gov/pubmed/33758507
http://dx.doi.org/10.2147/TCRM.S213020
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