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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 Oncogenes and Tumor Suppressors Free Research Articles Perspectives Clinical Trials and Observations Neoplasia 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. 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] 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]

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