Polycythemia Vera (PV): Update on Emerging Treatment Options

Giulia Benevolo, Francesco Vassallo, Irene Urbino, Valentina Giai Division of Haematology, Città della Salute e della Scienza, Turin, ItalyCorrespondence: Giulia BenevoloHematology, Città della Salute e della Scienza, Corso Bramante 88/90, Turin, 10126, ItalyEmail gbenevolo@cit...

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Autores principales: Benevolo G, Vassallo F, Urbino I, Giai V
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Publicado: Dove Medical Press 2021
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spelling oai:doaj.org-article:58310b20c1764f47a020ac0567c748962021-12-02T14:11:52ZPolycythemia Vera (PV): Update on Emerging Treatment Options1178-203Xhttps://doaj.org/article/58310b20c1764f47a020ac0567c748962021-03-01T00:00:00Zhttps://www.dovepress.com/polycythemia-vera-pv-update-on-emerging-treatment-options-peer-reviewed-article-TCRMhttps://doaj.org/toc/1178-203XGiulia Benevolo, Francesco Vassallo, Irene Urbino, Valentina Giai Division of Haematology, Città della Salute e della Scienza, Turin, ItalyCorrespondence: Giulia BenevoloHematology, Città della Salute e della Scienza, Corso Bramante 88/90, Turin, 10126, ItalyEmail gbenevolo@cittadellasalute.to.itAbstract: 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.Keywords: polycythemia vera, therapy, ruxolitinib, interferon, givinostatBenevolo GVassallo FUrbino IGiai VDove Medical Pressarticlepolycythemia veratherapyruxolitinibinterferongivinostatTherapeutics. PharmacologyRM1-950ENTherapeutics and Clinical Risk Management, Vol Volume 17, Pp 209-221 (2021)
institution DOAJ
collection DOAJ
language EN
topic polycythemia vera
therapy
ruxolitinib
interferon
givinostat
Therapeutics. Pharmacology
RM1-950
spellingShingle polycythemia vera
therapy
ruxolitinib
interferon
givinostat
Therapeutics. Pharmacology
RM1-950
Benevolo G
Vassallo F
Urbino I
Giai V
Polycythemia Vera (PV): Update on Emerging Treatment Options
description Giulia Benevolo, Francesco Vassallo, Irene Urbino, Valentina Giai Division of Haematology, Città della Salute e della Scienza, Turin, ItalyCorrespondence: Giulia BenevoloHematology, Città della Salute e della Scienza, Corso Bramante 88/90, Turin, 10126, ItalyEmail gbenevolo@cittadellasalute.to.itAbstract: 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.Keywords: polycythemia vera, therapy, ruxolitinib, interferon, givinostat
format article
author Benevolo G
Vassallo F
Urbino I
Giai V
author_facet Benevolo G
Vassallo F
Urbino I
Giai V
author_sort Benevolo G
title Polycythemia Vera (PV): Update on Emerging Treatment Options
title_short 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_sort polycythemia vera (pv): update on emerging treatment options
publisher Dove Medical Press
publishDate 2021
url https://doaj.org/article/58310b20c1764f47a020ac0567c74896
work_keys_str_mv AT benevolog polycythemiaverapvupdateonemergingtreatmentoptions
AT vassallof polycythemiaverapvupdateonemergingtreatmentoptions
AT urbinoi polycythemiaverapvupdateonemergingtreatmentoptions
AT giaiv polycythemiaverapvupdateonemergingtreatmentoptions
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