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Literature DB >> 29222296

What are the current treatment approaches for patients with polycythemia vera and essential thrombocythemia?

Alessandro M Vannucchi¹, Paola Guglielmelli¹.

Abstract

Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms that are characterized by thrombohemorrhagic complications, symptom burden, and impaired survival mainly due to thrombosis, progression to myelofibrosis, and transformation to acute leukemia. In this manuscript, we will review the most recent changes in diagnostic criteria, the improvements in risk stratification, and the "state of the art" in the daily management of these disorders. The role of conventional therapies and novel agents, interferon α and the JAK2 inhibitor ruxolitinib, is critically discussed based on the results of a few basic randomized clinical studies. Several unmet needs remain, above all, the lack of a curative approach that might overcome the still burdensome morbidity and mortality of these hematologic neoplasms, as well as the toxicities associated with therapeutic agents.

Entities: Chemical Disease Gene Species

Mesh：

Substances：

Year: 2017 PMID： 29222296 PMCID： PMC6142598 DOI： 10.1182/asheducation-2017.1.480

Source DB: PubMed Journal: Hematology Am Soc Hematol Educ Program ISSN： 1520-4383

49 in total

1. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.

Authors: A M Vannucchi; T Barbui; F Cervantes; C Harrison; J-J Kiladjian; N Kröger; J Thiele; C Buske
Journal: Ann Oncol Date: 2015-08-04 Impact factor: 32.976

2. Characteristics and treatment of polycythemia vera patients in clinical practice: a multicenter chart review on 1476 individuals in Germany.

Authors: Kathleen Jentsch-Ullrich; Judith Eberhardt; Vanja Zeremski; Michael Koehler; Denise Wolleschak; Florian H Heidel
Journal: J Cancer Res Clin Oncol Date: 2016-07-18 Impact factor: 4.553

3. Administration of direct oral anticoagulants in patients with myeloproliferative neoplasms.

Authors: Jean-Christophe Ianotto; Marie-Anne Couturier; Hubert Galinat; Dominique Mottier; Christian Berthou; Gaëlle Guillerm; Eric Lippert; Aurélien Delluc
Journal: Int J Hematol Date: 2017-06-16 Impact factor: 2.490

4. Antiplatelet therapy versus observation in low-risk essential thrombocythemia with a CALR mutation.

Authors: Alberto Alvarez-Larrán; Arturo Pereira; Paola Guglielmelli; Juan Carlos Hernández-Boluda; Eduardo Arellano-Rodrigo; Francisca Ferrer-Marín; Alimam Samah; Martin Griesshammer; Ana Kerguelen; Bjorn Andreasson; Carmen Burgaleta; Jiri Schwarz; Valentín García-Gutiérrez; Rosa Ayala; Pere Barba; María Teresa Gómez-Casares; Chiara Paoli; Beatrice Drexler; Sonja Zweegman; Mary F McMullin; Jan Samuelsson; Claire Harrison; Francisco Cervantes; Alessandro M Vannucchi; Carlos Besses
Journal: Haematologica Date: 2016-05-12 Impact factor: 9.941

5. Clinical evaluation of the European LeukaemiaNet criteria for clinicohaematological response and resistance/intolerance to hydroxycarbamide in essential thrombocythaemia.

Authors: Juan-Carlos Hernández-Boluda; Alberto Alvarez-Larrán; Montse Gómez; Anna Angona; Paula Amat; Beatriz Bellosillo; Luz Martínez-Avilés; Blanca Navarro; Anabel Teruel; Francisco Martínez-Ruiz; Carles Besses
Journal: Br J Haematol Date: 2010-11-18 Impact factor: 6.998

6. Ruxolitinib for the treatment of inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): a randomised, open-label, phase 3b study.

Authors: Francesco Passamonti; Martin Griesshammer; Francesca Palandri; Miklos Egyed; Giulia Benevolo; Timothy Devos; Jeannie Callum; Alessandro M Vannucchi; Serdar Sivgin; Caroline Bensasson; Mahmudul Khan; Nadjat Mounedji; Guray Saydam
Journal: Lancet Oncol Date: 2016-12-02 Impact factor: 41.316

7. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis.

Authors: S Cortelazzo; G Finazzi; M Ruggeri; O Vestri; M Galli; F Rodeghiero; T Barbui
Journal: N Engl J Med Date: 1995-04-27 Impact factor: 91.245

8. Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the International Working Group for Myelofibrosis Research and Treatment.

Authors: G Barosi; R A Mesa; J Thiele; F Cervantes; P J Campbell; S Verstovsek; B Dupriez; R L Levine; F Passamonti; J Gotlib; J T Reilly; A M Vannucchi; C A Hanson; L A Solberg; A Orazi; A Tefferi
Journal: Leukemia Date: 2007-08-30 Impact factor: 11.528

9. Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis.

Authors: Paola Guglielmelli; Annalisa Pacilli; Giada Rotunno; Elisa Rumi; Vittorio Rosti; Federica Delaini; Margherita Maffioli; Tiziana Fanelli; Alessandro Pancrazzi; Daniela Pietra; Silvia Salmoiraghi; Carmela Mannarelli; Annalisa Franci; Chiara Paoli; Alessandro Rambaldi; Francesco Passamonti; Giovanni Barosi; Tiziano Barbui; Mario Cazzola; Alessandro M Vannucchi
Journal: Blood Date: 2017-03-28 Impact factor: 25.476

10. Differences in treatment goals and perception of symptom burden between patients with myeloproliferative neoplasms (MPNs) and hematologists/oncologists in the United States: Findings from the MPN Landmark survey.

Authors: Ruben A Mesa; Carole B Miller; Maureen Thyne; James Mangan; Sara Goldberger; Salman Fazal; Xiaomei Ma; Wendy Wilson; Dilan C Paranagama; David G Dubinski; Ahmad Naim; Shreekant Parasuraman; John Boyle; John O Mascarenhas
Journal: Cancer Date: 2016-09-30 Impact factor: 6.860

5 in total

What are the current treatment approaches for patients with polycythemia vera and essential thrombocythemia?

1. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.

2. Characteristics and treatment of polycythemia vera patients in clinical practice: a multicenter chart review on 1476 individuals in Germany.

3. Administration of direct oral anticoagulants in patients with myeloproliferative neoplasms.

4. Antiplatelet therapy versus observation in low-risk essential thrombocythemia with a CALR mutation.

5. Clinical evaluation of the European LeukaemiaNet criteria for clinicohaematological response and resistance/intolerance to hydroxycarbamide in essential thrombocythaemia.

6. Ruxolitinib for the treatment of inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): a randomised, open-label, phase 3b study.

7. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis.

8. Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the International Working Group for Myelofibrosis Research and Treatment.

9. Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis.

10. Differences in treatment goals and perception of symptom burden between patients with myeloproliferative neoplasms (MPNs) and hematologists/oncologists in the United States: Findings from the MPN Landmark survey.

1. The comorbidity of acute ischemic stroke and splenic infarction resulting from essential thrombocythemia.

Review 2. The Myeloproliferative Neoplasm Landscape: A Patient's Eye View.

3. Anti-IL-6 cytokine treatment has no impact on elevated hematocrit or splenomegaly in a polycythemia vera mouse model.

Review 4. JAK2 Variant Signaling: Genetic, Hematologic and Immune Implication in Chronic Myeloproliferative Neoplasms.

Review 5. Advances in the Treatment of Polycythemia Vera: Trends in Disease Management.