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Prepublished online as a Blood First Edition Paper on June 21, 2002; DOI 10.1182/blood-2002-04-1199.

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Submitted April 22, 2002
Accepted May 30, 2002

The World Health Organization (WHO) classification of the myeloid neoplasms

James W Vardiman*, Nancy Lee Harris, and Richard D Brunning

Department of Pathology, University of Chicago, Chicago, IL, USA
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
Department of Pathology and Laboratory Medicine, University of Minnesota, Minneapolis, MN, USA

* Corresponding author; email: jvardima{at}uchospitals.edu.

A World Health Organization (WHO) classification of hematopoietic and lymphoid neoplasms has recently been published. This classification was developed through the collaborative efforts of the Society for Hematopathology, the European Association of Hematopathologists, and over 100 clinical hematologists and scientists who are internationally recognized for their expertise in hematopoietic neoplasms. For the lymphoid neoplasms, this classification provides a refinement of the entities described in the REAL classification -- a system that is now used worldwide. To date, however, there has been no published explanation or rationale given for the WHO classification of myeloid neoplasms. 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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L. Malcovati and M. Cazzola
Myelodysplastic/myeloproliferative disorders
Haematologica, January 1, 2008; 93(1): 4 - 6.
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haematolHome page
A. H. Schmitt-Graeff, S.-S. Teo, M. Olschewski, F. Schaub, S. Haxelmans, A. Kirn, P. Reinecke, U. Germing, and R. C. Skoda
JAK2V617F mutation status identifies subtypes of refractory anemia with ringed sideroblasts associated with marked thrombocytosis
Haematologica, January 1, 2008; 93(1): 34 - 40.
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haematolHome page
E. Antonioli, P. Guglielmelli, G. Poli, C. Bogani, A. Pancrazzi, G. Longo, V. Ponziani, L. Tozzi, L. Pieri, V. Santini, et al.
Influence of JAK2V617F allele burden on phenotype in essential thrombocythemia
Haematologica, January 1, 2008; 93(1): 41 - 48.
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ASH Education BookHome page
R. L. Levine and M. Heaney
New Advances in the Pathogenesis and Therapy of Essential Thrombocythemia
Hematology, January 1, 2008; 2008(1): 76 - 82.
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Cancer Res.Home page
N. Omidvar, S. Kogan, S. Beurlet, C. le Pogam, A. Janin, R. West, M.-E. Noguera, M. Reboul, A. Soulie, C. Leboeuf, et al.
BCL-2 and Mutant NRAS Interact Physically and Functionally in a Mouse Model of Progressive Myelodysplasia
Cancer Res., December 15, 2007; 67(24): 11657 - 11667.
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JCOHome page
E. Rumi, F. Passamonti, M. G. Della Porta, C. Elena, L. Arcaini, L. Vanelli, C. Del Curto, D. Pietra, E. Boveri, C. Pascutto, et al.
Familial Chronic Myeloproliferative Disorders: Clinical Phenotype and Evidence of Disease Anticipation
J. Clin. Oncol., December 10, 2007; 25(35): 5630 - 5635.
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BloodHome page
G. Barosi, G. Bergamaschi, M. Marchetti, A. M. Vannucchi, P. Guglielmelli, E. Antonioli, M. Massa, V. Rosti, R. Campanelli, L. Villani, et al.
JAK2 V617F mutational status predicts progression to large splenomegaly and leukemic transformation in primary myelofibrosis
Blood, December 1, 2007; 110(12): 4030 - 4036.
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haematolHome page
G. J.L. Kaspers and C. M. Zwaan
Pediatric acute myeloid leukemia: towards high-quality cure of all patients
Haematologica, November 1, 2007; 92(11): 1519 - 1532.
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BloodHome page
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.
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haematolHome page
F. Forghieri, M. Luppi, M. Morselli, L. Potenza, F. Volzone, G. Riva, A. Imovilli, E. Rivolti, and G. Torelli
Cytarabine-related lung infiltrates on high resolution computerized tomography: a possible complication with benign outcome in leukemic patients
Haematologica, September 1, 2007; 92(9): e85 - e90.
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BloodHome page
L. Bullinger, F. G. Rucker, S. Kurz, J. Du, C. Scholl, S. Sander, A. Corbacioglu, C. Lottaz, J. Krauter, S. Frohling, et al.
Gene-expression profiling identifies distinct subclasses of core binding factor acute myeloid leukemia
Blood, August 15, 2007; 110(4): 1291 - 1300.
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BloodHome page
C. Chang, B. E. Storer, B. L. Scott, E. M. Bryant, H. M. Shulman, M. E. Flowers, B. M. Sandmaier, R. P. Witherspoon, R. A. Nash, J. E. Sanders, et al.
Hematopoietic cell transplantation in patients with myelodysplastic syndrome or acute myeloid leukemia arising from myelodysplastic syndrome: similar outcomes in patients with de novo disease and disease following prior therapy or antecedent hematologic disorders
Blood, August 15, 2007; 110(4): 1379 - 1387.
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BloodHome page
A. Tefferi, J. Thiele, A. Orazi, H. M. Kvasnicka, T. Barbui, C. A. Hanson, G. Barosi, S. Verstovsek, G. Birgegard, R. Mesa, et al.
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
Blood, August 15, 2007; 110(4): 1092 - 1097.
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JCOHome page
L. Malcovati, U. Germing, A. Kuendgen, M. G. Della Porta, C. Pascutto, R. Invernizzi, A. Giagounidis, B. Hildebrandt, P. Bernasconi, S. Knipp, et al.
Time-Dependent Prognostic Scoring System for Predicting Survival and Leukemic Evolution in Myelodysplastic Syndromes
J. Clin. Oncol., August 10, 2007; 25(23): 3503 - 3510.
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BloodHome page
A. M. Vannucchi, E. Antonioli, P. Guglielmelli, A. Rambaldi, G. Barosi, R. Marchioli, R. M. Marfisi, G. Finazzi, V. Guerini, F. Fabris, et al.
Clinical profile of homozygous JAK2 617V>F mutation in patients with polycythemia vera or essential thrombocythemia
Blood, August 1, 2007; 110(3): 840 - 846.
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BloodHome page
F. Passamonti, M. L. Randi, E. Rumi, E. Pungolino, C. Elena, D. Pietra, M. Scapin, L. Arcaini, F. Tezza, R. Moratti, et al.
Increased risk of pregnancy complications in patients with essential thrombocythemia carrying the JAK2 (617V>F) mutation
Blood, July 15, 2007; 110(2): 485 - 489.
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M. A. Dawson, S. Avery, Z. K. McQuilten, M. J. Bailey, J. Shortt, M. N. Polizzotto, E. M. Wood, and M. F. Cole-Sinclair
Blood transfusion requirements for patients undergoing chemotherapy for acute myeloid leukemia how much is enough?
Haematologica, July 1, 2007; 92(7): 996 - 997.
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BloodHome page
A. Theocharides, M. Boissinot, F. Girodon, R. Garand, S.-S. Teo, E. Lippert, P. Talmant, A. Tichelli, S. Hermouet, and R. C. Skoda
Leukemic blasts in transformed JAK2-V617F-positive myeloproliferative disorders are frequently negative for the JAK2-V617F mutation.
Blood, July 1, 2007; 110(1): 375 - 379.
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BloodHome page
L. Teofili, M. Martini, T. Cenci, G. Petrucci, L. Torti, S. Storti, F. Guidi, G. Leone, and L. M. Larocca
Different STAT-3 and STAT-5 phosphorylation discriminates among Ph-negative chronic myeloproliferative diseases and is independent of the V617F JAK-2 mutation
Blood, July 1, 2007; 110(1): 354 - 359.
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BloodHome page
J. Zhang, Y. Liu, C. Beard, D. A. Tuveson, R. Jaenisch, T. E. Jacks, and H. F. Lodish
Expression of oncogenic K-ras from its endogenous promoter leads to a partial block of erythroid differentiation and hyperactivation of cytokine-dependent signaling pathways
Blood, June 15, 2007; 109(12): 5238 - 5241.
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BloodHome page
C. Gardin, P. Turlure, T. Fagot, X. Thomas, C. Terre, N. Contentin, E. Raffoux, S. de Botton, C. Pautas, O. Reman, et al.
Postremission treatment of elderly patients with acute myeloid leukemia in first complete remission after intensive induction chemotherapy:results of the multicenter randomized Acute Leukemia French Association (ALFA) 9803 trial
Blood, June 15, 2007; 109(12): 5129 - 5135.
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JCOHome page
E. Estey
Acute Myeloid Leukemia and Myelodysplastic Syndromes in Older Patients
J. Clin. Oncol., May 10, 2007; 25(14): 1908 - 1915.
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haematolHome page
A. L Basquiera, F. Fassetta, N. Soria, J. M. Barral, B. Ricchi, and J. J Garcia
Accuracy of leukocyte alkaline phosphatase score to predict JAK2 V617F mutation
Haematologica, May 1, 2007; 92(5): 704 - 705.
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J. Mol. Diagn.Home page
E. Hammond, K. Shaw, B. Carnley, S. P'ng, I. James, and R. Herrmann
Quantitative Determination of JAK2 V617F by TaqMan: An Absolute Measure of Averaged Copies per Cell That May Be Associated with the Different Types of Myeloproliferative Disorders
J. Mol. Diagn., April 1, 2007; 9(2): 242 - 248.
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JCOHome page
L. Teofili, F. Giona, M. Martini, T. Cenci, F. Guidi, L. Torti, G. Palumbo, A. Amendola, R. Foa, and L. M. Larocca
Markers of Myeloproliferative Diseases in Childhood Polycythemia Vera and Essential Thrombocythemia
J. Clin. Oncol., March 20, 2007; 25(9): 1048 - 1053.
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The OncologistHome page
S. Meshinchi and R. J. Arceci
Prognostic Factors and Risk-Based Therapy in Pediatric Acute Myeloid Leukemia
Oncologist, March 1, 2007; 12(3): 341 - 355.
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BloodHome page
J. E. Lancet, I. Gojo, J. Gotlib, E. J. Feldman, J. Greer, J. L. Liesveld, L. M. Bruzek, L. Morris, Y. Park, A. A. Adjei, et al.
A phase 2 study of the farnesyltransferase inhibitor tipifarnib in poor-risk and elderly patients with previously untreated acute myelogenous leukemia
Blood, February 15, 2007; 109(4): 1387 - 1394.
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ASH Education BookHome page
R. Skoda
The Genetic Basis of Myeloproliferative Disorders
Hematology, January 1, 2007; 2007(1): 1 - 10.
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A. M. Vannucchi and T. Barbui
Thrombocytosis and Thrombosis
Hematology, January 1, 2007; 2007(1): 363 - 370.
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ASH-SAPHome page
D. P. Steensma and R. E. Richard
Myeloproliferative disorders
ASH Self-Assessment Program, January 1, 2007; 2007(1): 172 - 227.
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BloodHome page
D. Grimwade
NPM1 mutation in AML: WHO and why?
Blood, December 15, 2006; 108(13): 3965 - 3965.
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