Journal ArticleDOI
Ropeginterferon alfa-2b versus standard therapy for polycythaemia vera (PROUD-PV and CONTINUATION-PV): a randomised, non-inferiority, phase 3 trial and its extension study
Heinz Gisslinger,Christoph Klade,Pencho Georgiev,Dorota Krochmalczyk,Liana Gercheva-Kyuchukova,Miklos Egyed,Viktor Rossiev,Petr Dulicek,Árpád Illés,Halyna Pylypenko,Lylia Sivcheva,Jiri Mayer,Vera Yablokova,Kurt Krejcy,Barbara Grohmann-Izay,Hans Carl Hasselbalch,Robert Kralovics,Jean-Jacques Kiladjian,Franz Bauer,Nicoleta Berbec,Carlos Besses Raebel,Zita Borbényi,Horia Bumbea,Veronika Buxhofer-Ausch,Malgorzata Calbecka,Emilie Cayssials-Caylus,Mario Cazzola,Olga Cerna,Andrei Cucuianu,Delia Dima,Ernst Forjan,Emanuil Gheorghita,Richard Greil,Antonia Hatalova,Mikulas Hrubisko,János Jakucs,Polina Kaplan,S V Klymenko,Steffen Koschmieder,Mihaela Lazaroiu,Tamila Lysa,Zvenyslava Masliak,Tamás Masszi,Georgi Mihaylov,Alexander Myasnikov,Uwe Platzbecker,Mathieu Puyade,Jerome Rey,Lydia Roy,Jiri Schwarz,Aleksander Skotnicki,Irina Sokolova,Maria Soroka-Wojtaszko,Jolanta Starzak-Gwozdz,Vera Stoeva,Jose Miguel Torregrosa-Diaz,Anna Vallova,Elena Volodicheva,Krzysztof Warzocha,Ella Willenbacher,Dominik Wolf +60 more
TLDR
The most frequently reported grade 3 and grade 4 treatment-related adverse events were increased γ-glutamyltransferase (seven [6%] of 127 patients) and increased alanine aminotransferase (four [3].About:
This article is published in The Lancet Haematology.The article was published on 2020-03-01. It has received 163 citations till now. The article focuses on the topics: Interim analysis & Tolerability.read more
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Journal ArticleDOI
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Ayalew Tefferi,Tiziano Barbui +1 more
TL;DR: Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms respectively characterized by clonal erythrocytosis and throm bocytosis; other disease features include leukocytotic features, splenomegaly, thromBosis, bleeding, microcirculatory symptoms, pruritus and risk of leukemic or fibrotic transformation.
Journal ArticleDOI
Ropeginterferon alfa-2b versus phlebotomy in low-risk patients with polycythaemia vera (Low-PV study): a multicentre, randomised phase 2 trial.
Tiziano Barbui,Alessandro M. Vannucchi,Valerio De Stefano,Arianna Masciulli,Alessandra Carobbio,Alberto Ferrari,Arianna Ghirardi,Elena Rossi,Fabio Ciceri,Massimiliano Bonifacio,Alessandra Iurlo,Francesca Palandri,Giulia Benevolo,Fabrizio Pane,Alessandra Ricco,Giuseppe Carli,Marianna Caramella,Davide Rapezzi,Caterina Musolino,Sergio Siragusa,Elisa Rumi,Andrea Patriarca,Nicola Cascavilla,Barbara Mora,Emma Cacciola,Carmela Mannarelli,Giuseppe Gaetano Loscocco,Paola Guglielmelli,Silvia Betti,Francesca Lunghi,Luigi Scaffidi,Cristina Bucelli,Nicola Vianelli,Marta Bellini,Maria Chiara Finazzi,Gianni Tognoni,Alessandro Rambaldi +36 more
TL;DR: The Low-PV study as mentioned in this paper compared the efficacy and safety of ropeginterferon alfa-2b on top of the standard phlebotomy regimen with PHLEbotomy alone.
Journal ArticleDOI
Interferon-alpha for treating polycythemia vera yields improved myelofibrosis-free and overall survival.
Ghaith Abu-Zeinah,Spencer Krichevsky,Tatiana Cruz,Gabriela Hoberman,Diana Jaber,Niamh Savage,Claudia Sosner,Ellen K. Ritchie,Joseph M. Scandura,Richard T. Silver +9 more
TL;DR: In this article, the authors compared the myelofibrosis-free survival (MFS) and overall survival (OS) with rIFNα to two other primary treatments, hydroxyurea (HU) and phlebotomy-only (PHL-O).
Posted ContentDOI
Phylogenetic reconstruction of myeloproliferative neoplasm reveals very early origins and lifelong evolution
Nicholas Williams,Joe Lee,Joe Lee,Luiza Moore,E. Joanna Baxter,James Hewinson,Kevin J. Dawson,Andrew Menzies,Anna L. Godfrey,Anthony R. Green,Anthony R. Green,Peter J. Campbell,Peter J. Campbell,Jyoti Nangalia +13 more
TL;DR: How driver mutation acquisition very early in life with life-long growth and evolution drive adult blood cancer is revealed, providing opportunities for early detection and intervention, and a new paradigm for cancer development.
Journal ArticleDOI
Molecular pathogenesis of the myeloproliferative neoplasms.
TL;DR: A comprehensive overview of the molecular pathophysiology of MPN exploring the role of driver mutations, co-occurring mutations, dysregulation of intrinsic cell signalling, epigenetic regulation and genetic predisposing factors highlighting important areas for future consideration as discussed by the authors.
References
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Journal ArticleDOI
The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes
James W. Vardiman,Juergen Thiele,Daniel A. Arber,Richard D. Brunning,Michael J. Borowitz,Anna Porwit,Nancy L. Harris,Michelle M. Le Beau,Eva Hellström-Lindberg,Ayalew Tefferi,Clara D. Bloomfield +10 more
TL;DR: The classification of myeloid neoplasms and acute leukemia is highlighted with the aim of familiarizing hematologists, clinical scientists, and hematopathologists not only with the major changes in the classification but also with the rationale for those changes.
Journal ArticleDOI
A Gain-of-Function Mutation of JAK2 in Myeloproliferative Disorders
Robert Kralovics,Francesco Passamonti,Andreas Buser,Soon Siong Teo,Ralph Tiedt,Jakob Passweg,André Tichelli,Mario Cazzola,Radek C. Skoda +8 more
TL;DR: Genetic evidence and in vitro functional studies indicate that V617F gives hematopoietic precursors proliferative and survival advantages and a high proportion of patients with myeloproliferative disorders carry a dominant gain-of-function mutation of JAK2.
Journal ArticleDOI
Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera
Jean-Jacques Kiladjian,Bruno Cassinat,Sylvie Chevret,Pascal Turlure,Nathalie Cambier,Murielle Roussel,Sylvia Bellucci,Bernard Grandchamp,Christine Chomienne,Pierre Fenaux +9 more
TL;DR: Results show that pegylated IFN-alpha-2a yields high rates of hematologic and molecular response in PV with limited toxicity, and could even eliminate the JAK2 mutated clone in selected cases.
Journal ArticleDOI
Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms
TL;DR: The genetic landscape of classical myeloproliferative neoplasm (MPN) is in large part elucidated and factors other than somatic mutations play an important role in disease initiation as well as disease progression such as germ line predisposition, inflammation, and aging.
Journal ArticleDOI
Pegylated Interferon Alfa-2a Yields High Rates of Hematologic and Molecular Response in Patients With Advanced Essential Thrombocythemia and Polycythemia Vera
Alfonso Quintás-Cardama,Hagop M. Kantarjian,Taghi Manshouri,Rajyalakshmi Luthra,Zeev Estrov,Sherry Pierce,Mary Ann Richie,Gautam Borthakur,Marina Konopleva,Jorge E. Cortes,Srdan Verstovsek +10 more
TL;DR: The ability of PEG-IFN-alpha-2a to induce complete molecular responses suggests selective targeting of the malignant clone in patients with advanced ET or PV.
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