Journal ArticleDOI
Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel.
Ayalew Tefferi,Juergen Thiele,Attilio Orazi,Hans Michael Kvasnicka,Tiziano Barbui,Curtis A. Hanson,Giovanni Barosi,Srdan Verstovsek,Gunnar Birgegård,Ruben A. Mesa,John T. Reilly,Heinz Gisslinger,Alessandro M. Vannucchi,Francisco Cervantes,Guido Finazzi,Ronald Hoffman,D. Gary Gilliland,Clara D. Bloomfield,James W. Vardiman +18 more
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TLDR
In this paper, the authors proposed a revision of the current World Health Organization (WHO) diagnostic criteria for polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), which was subsequently presented by an international expert panel of pathologists and clinical investigators in myeloproliferative disorders.About:
This article is published in Blood.The article was published on 2007-08-15. It has received 856 citations till now. The article focuses on the topics: Essential thrombocythemia & Myelofibrosis.read more
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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.
Journal ArticleDOI
Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms.
Ayalew Tefferi,James W. Vardiman +1 more
TL;DR: In 2008, the World Health Organization (WHO) treatise on the classification of hematopoietic tumors lists chronic myeloproliferative diseases (CMPDs) as a subdivision of myeloid neoplasms that includes the four classic myelopathological disorders (MPDs)-chronic myelogenous leukemia, polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), as well as chronic neutrophilic leukemia (CNL), chronic eos
Journal ArticleDOI
A dynamic prognostic model to predict survival in primary myelofibrosis: a study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment)
Francesco Passamonti,Francisco Cervantes,Alessandro M. Vannucchi,Enrica Morra,Elisa Rumi,Arturo Pereira,Paola Guglielmelli,Ester Pungolino,Marianna Caramella,Margherita Maffioli,Cristiana Pascutto,Mario Lazzarino,Mario Cazzola,Ayalew Tefferi +13 more
TL;DR: The novelty of these models is the prognostic assessment of patients with PMF anytime during their clinical course, which may be useful for treatment decision-making.
Journal ArticleDOI
Role of JAK2 in the pathogenesis and therapy of myeloproliferative disorders
TL;DR: The discoveries of JAK2V617F have changed the landscape for diagnosis and classification of PV, ET and PMF, and show the ability of genomic technologies to identify new molecular targets in human malignancies with pathogenetic, diagnostic and therapeutic significance.
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.
References
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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
Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders.
E. Joanna Baxter,Linda M. Scott,Peter J. Campbell,Clare L. East,Nasios Fourouclas,Soheila Swanton,George S. Vassiliou,Anthony J. Bench,Elaine M. Boyd,Natasha Curtin,Michael A. Scott,Wendy N. Erber,Anthony R. Green,Anthony R. Green +13 more
TL;DR: A single acquired mutation of JAK2 was noted in more than half of patients with a myeloproliferative disorder and its presence in all erythropoietin-independent erythroid colonies demonstrates a link with growth factor hypersensitivity, a key biological feature of these disorders.
Journal ArticleDOI
A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera
Chloé James,Valérie Ugo,Jean-Pierre Le Couedic,Judith Staerk,François Delhommeau,Catherine Lacout,Loïc Garçon,Hana Raslova,Roland Berger,Annelise Bennaceur-Griscelli,Jean-Luc Villeval,Stefan N. Constantinescu,Nicole Casadevall,William Vainchenker +13 more
TL;DR: A clonal and recurrent mutation in the JH2 pseudo-kinase domain of the Janus kinase 2 (JAK2) gene in most (> 80%) polycythaemia vera patients leads to constitutive tyrosine phosphorylation activity that promotes cytokine hypersensitivity and induces erythrocytosis in a mouse model.
Journal ArticleDOI
Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis.
Ross L. Levine,Ross L. Levine,Martha Wadleigh,Jan Cools,Benjamin L. Ebert,Benjamin L. Ebert,Gerlinde Wernig,Brian J. P. Huntly,Titus J. Boggon,Iwona Wlodarska,Jennifer J. Clark,Sandra A. Moore,Jennifer Adelsperger,Sumin Koo,Jeffrey C. Lee,Stacey Gabriel,Thomas Mercher,Alan D. D'Andrea,Stefan Fröhling,Konstanze Döhner,Peter Marynen,Peter Vandenberghe,Ruben A. Mesa,Ayalew Tefferi,James D. Griffin,Michael J. Eck,William R. Sellers,William R. Sellers,Matthew Meyerson,Matthew Meyerson,Todd R. Golub,Todd R. Golub,Todd R. Golub,Stephanie J. Lee,D. Gary Gilliland,D. Gary Gilliland,D. Gary Gilliland +36 more
TL;DR: High-throughput DNA resequencing identified a recurrent somatic missense mutation JAK2V617F in granulocyte DNA samples of 121 of 164 PV patients, of which 41 had homozygous and 80 had heterozygous mutations.
Journal ArticleDOI
The World Health Organization (WHO) classification of the myeloid neoplasms.
TL;DR: The purpose of this communication is to outline briefly the WHO classification of malignant myeloid diseases, to draw attention to major differences between it and antecedent classification schemes, and to provide the rationale for those differences.
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