SEARCH:   Advanced

Blood, 15 August 2007, Vol. 110, No. 4, pp. 1092-1097. Prepublished online as a Blood First Edition Paper on May 8, 2007; DOI 10.1182/blood-2007-04-083501. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2007-04-083501v1 110/4/1092    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Tefferi, A. Articles by Vardiman, J. W. Search for Related Content PubMed PubMed Citation Articles by Tefferi, A. Articles by Vardiman, J. W. Related Collections Perspectives Neoplasia Oncogenes and Tumor Suppressors Free Research Articles Clinical Trials and Observations Related Articles in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  PERSPECTIVE 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 Tefferi1, Juergen Thiele2, Attilio Orazi3, Hans Michael Kvasnicka2, Tiziano Barbui4, Curtis A. Hanson1, Giovanni Barosi5, Srdan Verstovsek6, Gunnar Birgegard7, Ruben Mesa1, John T. Reilly8, Heinz Gisslinger9, Alessandro M. Vannucchi10, Francisco Cervantes11, Guido Finazzi4, Ronald Hoffman12, D. Gary Gilliland13, Clara D. Bloomfield14, and James W. Vardiman15 1 Mayo Clinic, Rochester, MN; 2 Institute of Pathology, University of Cologne, Cologne, Germany; 3 Indiana University School of Medicine, Indianapolis; 4 Ospedali Riuniti di Bergamo, Bergamo, Italy; 5 Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico, San Matteo, Pavia, Italy; 6 M. D. Anderson Cancer Center, Houston, TX; 7 University Hospital, Uppsala, Sweden; 8 Royal Hallamshire Hospital, Sheffield, United Kingdom; 9 Medical University of Vienna, Vienna, Austria; 10 University of Florence, Florence, Italy; 11 Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain; 12 University of Illinois, Chicago; 13 Dana-Farber Cancer Institute, Boston, MA; 14 Ohio State University, Columbus; 15 University of Chicago, IL The Janus kinase 2 mutation, JAK2617V>F, is myeloid neoplasm-specific; its presence excludes secondary polycythemia, thrombocytosis, or bone marrow fibrosis from other causes. Furthermore, JAK2617V>F or a JAK2 exon 12 mutation is present in virtually all patients with polycythemia vera (PV), whereas JAK2617V>F also occurs in approximately half of patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF). Therefore, JAK2 mutation screening holds the promise of a decisive diagnostic test in PV while being complementary to histology for the diagnosis of ET and PMF; the combination of molecular testing and histologic review should also facilitate diagnosis of ET associated with borderline thrombocytosis. Accordingly, revision of the current World Health Organization (WHO) diagnostic criteria for PV, ET, and PMF is warranted; JAK2 mutation analysis should be listed as a major criterion for PV diagnosis, and the platelet count threshold for ET diagnosis can be lowered from 600 to 450 x 109/L. The current document was prepared by an international expert panel of pathologists and clinical investigators in myeloproliferative disorders; it was subsequently presented to members of the Clinical Advisory Committee for the revision of the WHO Classification of Myeloid Neoplasms, who endorsed the document and recommended its adoption by the WHO. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Articles in Blood Online: Revised criteria for the myeloproliferative disorders: too much too soon? Scott J. Samuelson, Charles J. Parker, and Josef T. Prchal Blood 2008 111: 1741. [Full Text] [PDF] Response: The 2008 World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: a paradigm of effective collaboration among clinicians, pathologists, and scientists Ayalew Tefferi, Juergen Thiele, and James W. Vardiman Blood 2008 111: 1742. [Full Text] [PDF] This article has been cited by other articles: J. Thiele, H. M. Kvasnicka, J. W. Vardiman, A. Orazi, V. Franco, H. Gisslinger, G. Birgegard, M. Griesshammer, and A. Tefferi Bone Marrow Fibrosis and Diagnosis of Essential Thrombocythemia J. Clin. Oncol., December 1, 2009; 27(34): e220 - e221. [Full Text] [PDF] L. Pieri, C. Bogani, P. Guglielmelli, M. Zingariello, R. A. Rana, N. Bartalucci, A. Bosi, and A. M. Vannucchi The JAK2V617 mutation induces constitutive activation and agonist hypersensitivity in basophils from patients with polycythemia vera Haematologica, November 1, 2009; 94(11): 1537 - 1545. [Abstract] [Full Text] [PDF] K. Kaya, R. Cavolli, A. Telli, and R. Tasoz Off-pump coronary artery surgery in a patient with essential thrombocythaemia: two life-threatening complications in the same patient BMJ Case Reports, August 25, 2009; 2009(aug25_1): bcr0120091503 - bcr0120091503. [Abstract] [Full Text] P. Guglielmelli, G. Barosi, G. Specchia, A. Rambaldi, F. Lo Coco, E. Antonioli, L. Pieri, A. Pancrazzi, V. Ponziani, F. Delaini, et al. Identification of patients with poorer survival in primary myelofibrosis based on the burden of JAK2V617F mutated allele Blood, August 20, 2009; 114(8): 1477 - 1483. [Abstract] [Full Text] [PDF] J. Thiele Philadelphia Chromosome-Negative Chronic Myeloproliferative Disease Am J Clin Pathol, August 1, 2009; 132(2): 261 - 280. [Abstract] [Full Text] [PDF] A. Orazi and M. B. Czader Myelodysplastic Syndromes Am J Clin Pathol, August 1, 2009; 132(2): 290 - 305. [Abstract] [Full Text] [PDF] F. Dentali, A. Squizzato, L. Brivio, L. Appio, L. Campiotti, M. Crowther, A. M. Grandi, and W. Ageno JAK2V617F mutation for the early diagnosis of Ph- myeloproliferative neoplasms in patients with venous thromboembolism: a meta-analysis Blood, May 28, 2009; 113(22): 5617 - 5623. [Abstract] [Full Text] [PDF] R. A. Mesa How I treat symptomatic splenomegaly in patients with myelofibrosis Blood, May 28, 2009; 113(22): 5394 - 5400. [Abstract] [Full Text] [PDF] S. Sozer, M. I. Fiel, T. Schiano, M. Xu, J. Mascarenhas, and R. Hoffman The presence of JAK2V617F mutation in the liver endothelial cells of patients with Budd-Chiari syndrome Blood, May 21, 2009; 113(21): 5246 - 5249. [Abstract] [Full Text] [PDF] G. Barosi, G. Birgegard, G. Finazzi, M. Griesshammer, C. Harrison, H. C. Hasselbalch, J.-J. Kiladjian, E. Lengfelder, M. F. McMullin, F. Passamonti, et al. Response criteria for essential thrombocythemia and polycythemia vera: result of a European LeukemiaNet consensus conference Blood, May 14, 2009; 113(20): 4829 - 4833. [Abstract] [Full Text] [PDF] V. De Stefano, T. Za, E. Rossi, A. Fiorini, A. Ciminello, C. Luzzi, P. Chiusolo, S. Sica, and G. Leone Influence of the JAK2 V617F mutation and inherited thrombophilia on the thrombotic risk among patients with essential thrombocythemia Haematologica, May 1, 2009; 94(5): 733 - 737. [Abstract] [Full Text] [PDF] A. M. Vannucchi, P. Guglielmelli, and A. Tefferi Advances in Understanding and Management of Myeloproliferative Neoplasms CA Cancer J Clin, May 1, 2009; 59(3): 171 - 191. [Abstract] [Full Text] [PDF] A M Florena, C Tripodo, A Di Bernardo, E Iannitto, C Guarnotta, R Porcasi, S Ingrao, V Abbadessa, and V Franco Different immunophenotypical apoptotic profiles characterise megakaryocytes of essential thrombocythaemia and primary myelofibrosis J. Clin. Pathol., April 1, 2009; 62(4): 331 - 338. [Abstract] [Full Text] [PDF] F. Cervantes, B. Dupriez, A. Pereira, F. Passamonti, J. T. Reilly, E. Morra, A. M. Vannucchi, R. A. Mesa, J.-L. Demory, G. Barosi, et al. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment Blood, March 26, 2009; 113(13): 2895 - 2901. [Abstract] [Full Text] [PDF] I. Rapado, S. Grande, E. Albizua, R. Ayala, J.-A. Hernandez, M. Gallardo, F. Gilsanz, and J. Martinez-Lopez High Resolution Melting Analysis for JAK2 Exon 14 and Exon 12 Mutations: A Diagnostic Tool for Myeloproliferative Neoplasms J. Mol. Diagn., March 1, 2009; 11(2): 155 - 161. [Abstract] [Full Text] [PDF] S. Schnittger, U. Bacher, C. Haferlach, T. Geer, P. Muller, J. Mittermuller, P. Petrides, R. Schlag, R. Sandner, J. Selbach, et al. Detection of JAK2 exon 12 mutations in 15 patients with JAK2V617F negative polycythemia vera Haematologica, March 1, 2009; 94(3): 414 - 418. [Abstract] [Full Text] [PDF] J. Thiele and H. M. Kvasnicka Myelofibrotic transformation in essential thrombocythemia Haematologica, March 1, 2009; 94(3): 431 - 433. [Full Text] [PDF] F. Passamonti, E. Rumi, E. Boveri, and M. Lazzarino Myelofibrotic transformation in essential thrombocythemia. Author reply Haematologica, March 1, 2009; 94(3): 433 - 433. [Full Text] [PDF] M. G. Della Porta, L. Malcovati, E. Boveri, E. Travaglino, D. Pietra, C. Pascutto, F. Passamonti, R. Invernizzi, A. Castello, U. Magrini, et al. Clinical Relevance of Bone Marrow Fibrosis and CD34-Positive Cell Clusters in Primary Myelodysplastic Syndromes J. Clin. Oncol., February 10, 2009; 27(5): 754 - 762. [Abstract] [Full Text] [PDF] V. Seaman, A. Jumaan, E. Yanni, B. Lewis, J. Neyer, P. Roda, M. Xu, and R. Hoffman Use of Molecular Testing to Identify a Cluster of Patients with Polycythemia Vera in Eastern Pennsylvania Cancer Epidemiol. Biomarkers Prev., February 1, 2009; 18(2): 534 - 540. [Abstract] [Full Text] [PDF] J. Malysz and D. Crisan Correlation of JAK2 V617F Mutant Allele Quantitation with Clinical Presentation and Type of Chronic Myeloproliferative Neoplasm Ann. Clin. Lab. Sci., January 1, 2009; 39(4): 345 - 350. [Abstract] [Full Text] [PDF] P. Guglielmelli, G. Barosi, L. Pieri, E. Antonioli, A. Bosi, and A. M. Vannucchi JAK2V617F mutational status and allele burden have little influence on clinical phenotype and prognosis in patients with post-polycythemia vera and post-essential thrombocythemia myelofibrosis Haematologica, January 1, 2009; 94(1): 144 - 146. [Full Text] [PDF] F. Passamonti, E. Rumi, L. Arcaini, E. Boveri, C. Elena, D. Pietra, S. Boggi, C. Astori, P. Bernasconi, M. Varettoni, et al. Prognostic factors for thrombosis, myelofibrosis, and leukemia in essential thrombocythemia: a study of 605 patients Haematologica, November 1, 2008; 93(11): 1645 - 1651. [Abstract] [Full Text] [PDF] S. I. Swierczek, N. Agarwal, R. H. Nussenzveig, G. Rothstein, A. Wilson, A. Artz, and J. T. Prchal Hematopoiesis is not clonal in healthy elderly women Blood, October 15, 2008; 112(8): 3186 - 3193. [Abstract] [Full Text] [PDF] J.-J. Kiladjian, B. Cassinat, S. Chevret, P. Turlure, N. Cambier, M. Roussel, S. Bellucci, B. Grandchamp, C. Chomienne, and P. Fenaux Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera Blood, October 15, 2008; 112(8): 3065 - 3072. [Abstract] [Full Text] [PDF] A. V. Jones, N. C.P. Cross, H. E. White, A. R. Green, and L. M. Scott Rapid identification of JAK2 exon 12 mutations using high resolution melting analysis Haematologica, October 1, 2008; 93(10): 1560 - 1564. [Abstract] [Full Text] [PDF] A. Pancrazzi, P. Guglielmelli, V. Ponziani, G. Bergamaschi, A. Bosi, G. Barosi, and A. M. Vannucchi A Sensitive Detection Method for MPLW515L or MPLW515K Mutation in Chronic Myeloproliferative Disorders with Locked Nucleic Acid-Modified Probes and Real-Time Polymerase Chain Reaction J. Mol. Diagn., September 1, 2008; 10(5): 435 - 441. [Abstract] [Full Text] [PDF] I Mirza, D Sekora, and C Frantz Testing for JAK2V617F mutation across specimen types yields concordant results J. Clin. Pathol., August 1, 2008; 61(8): 975 - 975. [Full Text] [PDF] A. M. Vannucchi, E. Antonioli, P. Guglielmelli, A. Pancrazzi, V. Guerini, G. Barosi, M. Ruggeri, G. Specchia, F. Lo-Coco, F. Delaini, et al. Characteristics and clinical correlates of MPL 515W>L/K mutation in essential thrombocythemia Blood, August 1, 2008; 112(3): 844 - 847. [Abstract] [Full Text] [PDF] J. L. Spivak and R. T. Silver The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal Blood, July 15, 2008; 112(2): 231 - 239. [Full Text] [PDF] A. M. Vannucchi and P. Guglielmelli Molecular pathophysiology of Philadelphia-negative myeloproliferative disorders: beyond JAK2 and MPL mutations Haematologica, July 1, 2008; 93(7): 972 - 976. [Full Text] [PDF] O. Bock, J. Hoftmann, K. Theophile, K. Hussein, B. Wiese, J. Schlue, and H. Kreipe Bone Morphogenetic Proteins Are Overexpressed in the Bone Marrow of Primary Myelofibrosis and Are Apparently Induced by Fibrogenic Cytokines Am. J. Pathol., April 1, 2008; 172(4): 951 - 960. [Abstract] [Full Text] [PDF] S. Li, R. Kralovics, G. De Libero, A. Theocharides, H. Gisslinger, and R. C. Skoda Clonal heterogeneity in polycythemia vera patients with JAK2 exon12 and JAK2-V617F mutations Blood, April 1, 2008; 111(7): 3863 - 3866. [Abstract] [Full Text] [PDF] K. Ohyashiki, T. Kiguchi, Y. Ito, A. Gotoh, T. Tauchi, K. Miyazawa, Y. Kimura, H. Hisatomi, and J. H. Ohyashiki Isolated Erythrocythemia: A Distinct Entity or a Sub-type of Polycythemia Vera? Jpn. J. Clin. Oncol., March 1, 2008; 38(3): 230 - 232. [Full Text] [PDF] L. Teofili, R. Foa, F. Giona, and L. M. Larocca Childhood polycythemia vera and essential thrombocythemia: does their pathogenesis overlap with that of adult patients? Haematologica, February 1, 2008; 93(2): 169 - 172. [Full Text] [PDF] S. J. Samuelson, C. J. Parker, and J. T. Prchal Revised criteria for the myeloproliferative disorders: too much too soon? Blood, February 1, 2008; 111(3): 1741 - 1741. [Full Text] [PDF] A. Tefferi, J. Thiele, and J. W. Vardiman Response: The 2008 World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: a paradigm of effective collaboration among clinicians, pathologists, and scientists Blood, February 1, 2008; 111(3): 1742 - 1742. [Full Text] [PDF] D. Pietra, S. Li, A. Brisci, F. Passamonti, E. Rumi, A. Theocharides, M. Ferrari, H. Gisslinger, R. Kralovics, L. Cremonesi, et al. Somatic mutations of JAK2 exon 12 in patients with JAK2 (V617F)-negative myeloproliferative disorders Blood, February 1, 2008; 111(3): 1686 - 1689. [Abstract] [Full Text] [PDF] C. James The JAK2V617F Mutation in Polycythemia Vera and Other Myeloproliferative Disorders: One Mutation for Three Diseases? Hematology, January 1, 2008; 2008(1): 69 - 75. [Abstract] [Full Text] [PDF] R. L. Levine and M. Heaney New Advances in the Pathogenesis and Therapy of Essential Thrombocythemia Hematology, January 1, 2008; 2008(1): 76 - 82. [Abstract] [Full Text] [PDF] L. Peterson Prefibrotic myelofibrosis: is this diagnosis valid? Blood, January 1, 2008; 111(1): 1 - 2. [Full Text] [PDF] J. W. Vardiman Integration of Histology and Genetics in the Diagnosis and Classification of Myeloproliferative Neoplasms ASCO Educational Book, January 1, 2008; 2008(1): 339 - 345. [Abstract] [Full Text] [PDF] A. Tefferi Management of Myeloproliferative Neoplasms and the Promise of Targeted Therapy ASCO Educational Book, January 1, 2008; 2008(1): 346 - 348. [Abstract] [Full Text] [PDF] B. S. Wilkins, W. N. Erber, D. Bareford, G. Buck, K. Wheatley, C. L. East, B. Paul, C. N. Harrison, A. R. Green, and P. J. Campbell Bone marrow pathology in essential thrombocythemia: interobserver reliability and utility for identifying disease subtypes Blood, January 1, 2008; 111(1): 60 - 70. [Abstract] [Full Text] [PDF] E. Antonioli, P. Guglielmelli, G. Poli, V. Santini, A. Bosi, and A. M. Vannucchi Polycythemia vera following autologous transplantation for AML: insights on the kinetics of JAK2V617F clonal dominance Blood, December 15, 2007; 110(13): 4620 - 4621. [Full Text] [PDF] M. Cazzola Somatic mutations of JAK2 exon 12 as a molecular basis of erythrocytosis Haematologica, December 1, 2007; 92(12): 1585 - 1589. [Full Text] [PDF] L. Teofili, F. Giona, M. Martini, T. Cenci, F. Guidi, L. Torti, G. Palumbo, A. Amendola, G. Leone, R. Foa, et al. The revised WHO diagnostic criteria for Ph-negative myeloproliferative diseases are not appropriate for the diagnostic screening of childhood polycythemia vera and essential thrombocythemia Blood, November 1, 2007; 110(9): 3384 - 3386. [Abstract] [Full Text] [PDF] R. Skoda The Genetic Basis of Myeloproliferative Disorders Hematology, January 1, 2007; 2007(1): 1 - 10. [Abstract] [Full Text] [PDF] R. Hoffman and D. Rondelli Biology and Treatment of Primary Myelofibrosis Hematology, January 1, 2007; 2007(1): 346 - 354. [Abstract] [Full Text] [PDF] R. A. Mesa Navigating the Evolving Paradigms in the Diagnosis and Treatment of Myeloproliferative Disorders Hematology, January 1, 2007; 2007(1): 355 - 362. [Abstract] [Full Text] [PDF] A. M. Vannucchi and T. Barbui Thrombocytosis and Thrombosis Hematology, January 1, 2007; 2007(1): 363 - 370. [Abstract] [Full Text] [PDF]

	Copyright © 2007 by American Society of Hematology         Online ISSN: 1528-0020