Philadelphia-Negative Classical Myeloproliferative Neoplasms: Critical Concepts and Management Recommendations From European LeukemiaNet
Tiziano Barbui,Giovanni Barosi,Gunnar Birgegård,Francisco Cervantes,Guido Finazzi,Martin Griesshammer,Claire N. Harrison,Hans Carl Hasselbalch,Rüdiger Hehlmann,Ronald Hoffman,Jean-Jacques Kiladjian,Kröger N,Ruben A. Mesa,Mary Frances McMullin,Animesh Pardanani,Francesco Passamonti,Alessandro M. Vannucchi,Andreas Reiter,Richard T. Silver,Srdan Verstovsek,Ayalew Tefferi +20 more
TLDR
A review of critical concepts and recommendations on the management of Philadelphia-negative classical myeloproliferative neoplasms, including monitoring, response definition, first- and second-line therapy, and therapy for special issues are presented.Abstract:
We present a review of critical concepts and produce recommendations on the management of Philadelphia-negative classical myeloproliferative neoplasms, including monitoring, response definition, fi ...read more
Citations
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Journal ArticleDOI
Somatic CALR Mutations in Myeloproliferative Neoplasms with Nonmutated JAK2
Jyoti Nangalia,Charles E. Massie,E J Baxter,Francesca L. Nice,Gunes Gundem,David C. Wedge,Edward Avezov,Juan Li,Karoline Kollmann,David G. Kent,Athar Aziz,Anna L. Godfrey,Jonathon Hinton,Inigo Martincorena,P Van Loo,Amy V. Jones,Paola Guglielmelli,P. S. Tarpey,Heather P. Harding,J.D. Fitzpatrick,C.T. Goudie,Christina A. Ortmann,Stephen J. Loughran,Keiran Raine,David R. Jones,Adam Butler,Jon W. Teague,Sarah O’Meara,Stuart McLaren,M. Bianchi,Yvonne Silber,D. Dimitropoulou,David Bloxham,L. Mudie,Mark Maddison,Bruce W. S. Robinson,Clodagh Keohane,Cathy MacLean,Kate Hill,Kim Orchard,Sudhir Tauro,Ming-Qing Du,Mel Greaves,David G. Bowen,Brian J. P. Huntly,Claire N. Harrison,Nicholas C.P. Cross,David Ron,Alessandro M. Vannucchi,Elli Papaemmanuil,Peter J. Campbell,Anthony R. Green +51 more
TL;DR: Somatic mutations in the endoplasmic reticulum chaperone CALR were found in a majority of patients with myeloproliferative neoplasms with nonmutated JAK2, a finding consistent with its role as an initiating mutation in some patients.
Journal ArticleDOI
Ruxolitinib versus Standard Therapy for the Treatment of Polycythemia Vera
Alessandro M. Vannucchi,Jean-Jacques Kiladjian,Martin Griesshammer,Tamás Masszi,Simon Durrant,Francesco Passamonti,Claire N. Harrison,Fabrizio Pane,Pierre Zachee,Ruben A. Mesa,Shui He,Mark M. Jones,William M. Garrett,Jingjin Li,Ulrich Pirron,Dany Habr,Srdan Verstovsek +16 more
TL;DR: In patients who had an inadequate response to or had unacceptable side effects from hydroxyurea, ruxolitinib was superior to standard therapy in controlling the hematocrit, reducing the spleen volume, and improving symptoms associated with polycythemia vera.
Journal ArticleDOI
Cardiovascular Events and Intensity of Treatment in Polycythemia Vera
Roberto Marchioli,Guido Finazzi,Giorgina Specchia,Rossella R. Cacciola,Riccardo Cavazzina,Daniela Cilloni,Valerio De Stefano,Elena Maria Elli,Alessandra Iurlo,Roberto Latagliata,Francesca Lunghi,Monia Lunghi,Rosa Maria Marfisi,Pellegrino Musto,Arianna Masciulli,Caterina Musolino,Nicola Cascavilla,Giovanni Quarta,Maria Luigia Randi,Davide Rapezzi,Marco Ruggeri,Elisa Rumi,Anna Rita Scortechini,Simone Santini,Marco Scarano,Sergio Siragusa,Antonio Spadea,Alessia Tieghi,Emanuele Angelucci,Giuseppe Visani,Alessandro M. Vannucchi,Tiziano Barbui +31 more
TL;DR: In patients with polycythemia vera, those with a hematocrit target of less than 45% had a significantly lower rate of cardiovascular death and major thrombosis than did those with an intended target of 45 to 50%.
Journal ArticleDOI
JAK2 or CALR mutation status defines subtypes of essential thrombocythemia with substantially different clinical course and outcomes
Elisa Rumi,Daniela Pietra,Virginia Valeria Ferretti,Thorsten Klampfl,Ashot S. Harutyunyan,Jelena D. Milosevic,Nicole C.C. Them,Tiina Berg,Chiara Elena,Ilaria Carola Casetti,Chiara Milanesi,Emanuela Sant'Antonio,Marta Bellini,Elena Fugazza,Maria C. Renna,Emanuela Boveri,Cesare Astori,Cristiana Pascutto,Robert Kralovics,Robert Kralovics,Mario Cazzola +20 more
TL;DR: Observations are consistent with the notion that JAK2-mutated essential thrombocythemia and polycythemia vera represent different phenotypes of a single myeloproliferative neoplasm, whereas CALR-mutation essential thROMbocyhimia is a distinct disease entity.
Journal ArticleDOI
Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis)
Tiziano Barbui,Guido Finazzi,Alessandra Carobbio,Juergen Thiele,Francesco Passamonti,Elisa Rumi,Marco Ruggeri,Francesco Rodeghiero,Maria Luigia Randi,Irene Bertozzi,Heinz Gisslinger,Veronika Buxhofer-Ausch,Valerio De Stefano,Silvia Betti,Alessandro Rambaldi,Alessandro M. Vannucchi,Ayalew Tefferi +16 more
TL;DR: The 3-tiered new prognostic model, devised in an international study of 891 patients with essential thrombocythemia, outperformed conventional risk stratification in predicting future vascular events.
References
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WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
TL;DR: Thank you very much for reading who classification of tumours of haematopoietic and lymphoid tissues, and maybe you have knowledge that, people have look hundreds of times for their chosen readings like this, but end up in malicious downloads.
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Guidelines for the diagnosis and treatment of pulmonary hypertension
Marius M. Hoeper,Marc Humbert,Adam Torbicki,Jean-Luc Vachiery,Joan Albert Barberà,Maurice Beghetti,Paul A. Corris,Sean Gaine,J. Simon R. Gibbs,Miguel Ángel Gómez-Sánchez,Guillaume Jondeau,Walter Klepetko,Andrew J. Peacock,Lewis J. Rubin,Michael J. Zellweger,Gérald Simonneau,Angelo Auricchio,Jeroen J. Bax,Claudio Ceconi,Veronica Dean,Gerasimos Filippatos,Christian Funck-Brentano,Richard J. Hobbs,Peter Kearney,Theresa McDonagh,Keith McGregor,Bogdan A. Popescu,Zeljko Reiner,Udo Sechtem,Michal Tendera,Panos E. Vardas,Petr Widimsky,Felicita Andreotti,Michael Aschermann,Riccardo Asteggiano,Ray Benza,Rolf M. F. Berger,Damien Bonnet,Marion Delcroix,Luke Howard,Anastasia Kitsiou,Irene Lang,Aldo P. Maggioni,Jens Erik Nielsen-Kudsk,Myung H. Park,Pasquale Perrone-Filardi,Maria Teresa Subirana Domenech,Anton Vonk-Noordegraaf,José Luis Zamorano +48 more
TL;DR: Guidelines and Expert Consensus Documents summarise and evaluate all currently available evidence on a particular issue with the aim to assist physicians in selecting the best management strategies for a typical patient, suffering from a given condition, taking into account the impact on outcome, as well as the risk/benefit ratio of particular diagnostic or therapeutic means.
Journal ArticleDOI
Guidelines for the diagnosis and treatment of pulmonary hypertension
Nazzareno Galiè,Marius M. Hoeper,Marc Humbert,Adam Torbicki,Jean-Luc Vachiery,Joan Albert Barberà,Maurice Beghetti,Paul A. Corris,Sean Gaine,J. Simon R. Gibbs,Miguel Ángel Gómez-Sánchez,Guillaume Jondeau,Walter Klepetko,Christian Opitz,Andrew J. Peacock,Lewis J. Rubin,Michael J. Zellweger,Gérald Simonneau +17 more
Journal ArticleDOI
New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment.
Francisco Cervantes,Brigitte Dupriez,Arturo Pereira,Francesco Passamonti,John T. Reilly,Enrica Morra,Alessandro M. Vannucchi,Ruben A. Mesa,Jean Loup Demory,Giovanni Barosi,Elisa Rumi,Ayalew Tefferi +11 more
TL;DR: In 409 patients with assessable metaphases, cytogenetic abnormalities were associated with shorter survival, but their independent contribution to prognosis was restricted to patients in the intermediate-risk groups.
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