International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data
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The classification of myeloid neoplasms and acute leukemias was last updated in 2016 within a collaboration between the World Health Organization (WHO), the Society for Hematopathology, and the European Association for Haematopathology. This collaboration was primarily based on input from a clinical advisory committees (CACs) composed of pathologists, hematologists, oncologists, geneticists, and bioinformaticians from around the world. The recent advances in our understanding of the biology of hematologic malignancies, the experience with the use of the 2016 WHO classification in clinical practice, and the results of clinical trials have indicated the need for further revising and updating the classification. As a continuation of this CAC-based process, the authors, a group with expertise in the clinical, pathologic, and genetic aspects of these disorders, developed the International Consensus Classification (ICC) of myeloid neoplasms and acute leukemias. Using a multiparameter approach, the main objective of the consensus process was the definition of real disease entities, including the introduction of new entities and refined criteria for existing diagnostic categories, based on accumulated data. The ICC is aimed at facilitating diagnosis and prognostication of these neoplasms, improving treatment of affected patients, and allowing the design of innovative clinical trials.
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Self-citationZanotti, R., Bonifacio, M., Lucchini, G., Sperr, W. R., Scaffidi, L., van Anrooij, B., Oude Elberink, H. N., Rossignol, J., Hermine, O., Gorska, A., et al. (2022) · Leukemia · doi:10.1038/s41375-021-01406-y · Cited by 56 · see citing works
Self-citationERCC6L2 defines a novel entity within inherited acute myeloid leukemia
Douglas, S. P. M., Siipola, P., Kovanen, P. E., Pyörälä, M., Kakko, S., Savolainen, E., Salmenniemi, U., Orte, K. J., Kytölä, S. I., Pitkänen, E., et al. (2019) · Blood · doi:10.1182/blood-2019-01-896233 · Cited by 55 · see citing works
Self-citationCytopenia levels for aiding establishment of the diagnosis of myelodysplastic syndromes
Greenberg, P. L., Tuechler, H., Schanz, J., Sanz, G. F., Garcia‐Manero, G., Solé, F. G., Bennett, J. M., Bowen, D., Fenaux, P., Dreyfus, F., et al. (2016) · Blood · doi:10.1182/blood-2016-07-728766 · Cited by 51 · see citing works
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Self-citationFamilial myeloid malignancies with germline TET2 mutation
Duployez, N., Goursaud, L., Fenwarth, L., Bories, C., Marceau‐Renaut, A., Boyer, T., Fournier, É., Nibourel, O., Roche‐Lestienne, C., Huet, G., et al. (2019) · Leukemia · doi:10.1038/s41375-019-0675-6 · Cited by 51 · see citing works
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Self-citationWang, S. A., Galili, N., Černý, J., Sechman, E. V., Chen, S. S., Loew, J. M., Liu, Q., Fadare, O., Hasserjian, R. P., Jones, D. M., et al. (2006) · American Journal of Clinical Pathology · doi:10.1309/fu04-p779-u310-r3ee · Cited by 49 · see citing works
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Prefibrotic myelofibrosis: treatment algorithm 2018
Finazzi, G., Vannucchi, A. M., & Barbui, T. (2018) · Blood Cancer Journal · doi:10.1038/s41408-018-0142-z · Cited by 49 · see citing works
Self-citationFend, F., Horn, T., Koch, I., Vela, T., & Orazi, A. (2008) · Leukemia research : a Forum for Studies on Leukemia and Normal Hemopoiesis · doi:10.1016/j.leukres.2008.04.024 · Cited by 48 · see citing works
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Wintering, A., Dvorak, C. C., Stieglitz, E., & Loh, M. L. (2021) · Blood Advances · doi:10.1182/bloodadvances.2021005117 · Cited by 47 · see citing works
Self-citationDíaz‐Beyá, M., Rozman, M., Pratcorona, M., Torrebadell, M., Camós, M., Aguilar, J. L., & Esteve, J. (2010) · Blood · doi:10.1182/blood-2010-09-307314 · Cited by 46 · see citing works
Self-citationDetection of NPM/MLF1 fusion in t(3;5)-positive acute myeloid leukemia and myelodysplasia
Arber, D. A., Chang, K. L., Lyda, M., et al. (2003) · Human Pathology · doi:10.1016/s0046-8177(03)00251-x · Cited by 46 · see citing works
Self-citationValidation of the 2017 revision of the WHO chronic myelomonocytic leukemia categories
Loghavi, S., Sui, D., Wei, P., Garcia‐Manero, G., Pierce, S. A., Routbort, M. J., Jabbour, E. J., Pemmaraju, N., Kanagal‐Shamanna, R., Gur, H. D., et al. (2018) · Blood Advances · doi:10.1182/bloodadvances.2018019224 · Cited by 46 · see citing works
Self-citationIdentification of FOXP1 and SNX2 as novel ABL1 fusion partners in acute lymphoblastic leukaemia
Ernst, T., Score, J., Deininger, M. W., Hidalgo-Curtis, C., Lackie, P. M., Ershler, W. B., Goldman, J. M., Cross, N. C. P., & Grand, F. (2011) · British Journal of Haematology · doi:10.1111/j.1365-2141.2010.08457.x · Cited by 45 · see citing works
Heterozygous germ line CSF3R variants as risk alleles for development of hematologic malignancies
Trottier, A. M., Druhan, L. J., Kraft, I. L., et al. (2020) · Blood Advances · doi:10.1182/bloodadvances.2020002013 · Cited by 45 · see citing works
Calvo, X., García-Gisbert, N., Párraga, I., Gibert, J., Florensa, L., Andrade‐Campos, M., Merchán, B., Garcia-Ávila, S., Montesdeoca, S., Fernández‐Rodríguez, C., et al. (2020) · Blood Advances · doi:10.1182/bloodadvances.2020002206 · Cited by 45 · see citing works
Haferlach, C., Bacher, U., Haferlach, T., et al. (2011) · Leukemia · doi:10.1038/leu.2011.5 · Cited by 44 · see citing works
Nucleophosmin 1 (NPM1) mutations in chronic myelomonocytic leukemia and their prognostic relevance
Vallapureddy, R. R., Lasho, T. L., Hoversten, K. P., et al. (2017) · American Journal of Hematology · doi:10.1002/ajh.24861 · Cited by 44 · see citing works
Szuber, N., Finke, C. M., Lasho, T. L., et al. (2018) · Blood Cancer Journal · doi:10.1038/s41408-018-0058-7 · Cited by 44 · see citing works
Sarasin, A. R., Quentin, S., Droin, N. M., et al. (2019) · Blood · doi:10.1182/blood-2019-01-895698 · Cited by 44 · see citing works
Myeloid/lymphoid neoplasms with FLT3 rearrangement
Tang, G., Tam, W., Short, N. J., et al. (2021) · Modern Pathology · doi:10.1038/s41379-021-00817-7 · Cited by 43 · see citing works
Gao, Y., Jia, M.-N., Mao, Y., Cai, H., Jiang, X., Cao, X., Zhou, D., & Li, J. (2021) · American Journal of Clinical Pathology · doi:10.1093/ajcp/aqab172 · Cited by 43 · see citing works
NPM1-Mutated Myeloid Neoplasms with <20% Blasts: A Really Distinct Clinico-Pathologic Entity?
Forghieri, F., Nasillo, V., Paolini, A., Bettelli, F., Pioli, V., Giusti, D., Gilioli, A., Colasante, C., Acquaviva, G., Riva, G., et al. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21238975 · Cited by 42 · see citing works
Idiopathic hypereosinophilia is clonal disorder? Clonality identified by targeted sequencing
Lee, J.-S., Seo, H., Im, K., et al. (2017) · PLoS ONE · doi:10.1371/journal.pone.0185602 · Cited by 41 · see citing works
Wang, S. A., Galili, N., Černý, J., et al. (2006) · American Journal of Clinical Pathology · doi:10.1309/fu04p779u310r3ee · Cited by 41 · see citing works
Self-citationFlorian, S., Esterbauer, H., Binder, T. M., Müllauer, L., Haas, O. A., Sperr, W. R., Sillaber, C., & Valent, P. (2006) · Leukemia Research · doi:10.1016/j.leukres.2005.11.014 · Cited by 40 · see citing works
Yoshimi, A., van den Heuvel‐Eibrink, M. M., Baumann, I., et al. (2013) · Haematologica · doi:10.3324/haematol.2013.095786 · Cited by 39 · see citing works
Giacomo, D. D., Starza, R. L., Gorello, P., Pellanera, F., Atak, Z. K., Keersmaecker, K. D., Pierini, V., Harrison, C. J., Arniani, S., Moretti, M., et al. (2021) · Blood · doi:10.1182/blood.2020010510 · Cited by 39 · see citing works
Bhavsar, S., Liu, Y.-C., Gibson, S. E., Moore, E. M., & Swerdlow, S. H. (2021) · The American Journal of Surgical Pathology · doi:10.1097/pas.0000000000001750 · Cited by 39 · see citing works
Weinberg, O. K., Pozdnyakova, O., Campigotto, F., DeAngelo, D. J., Stone, R. M., Neuberg, D. S., & Hasserjian, R. P. (2015) · Modern Pathology · doi:10.1038/modpathol.2015.55 · Cited by 38 · see citing works
Self-citationBuijs, A., & Bruin, M. C. A. (2007) · Leukemia · doi:10.1038/sj.leu.2404596 · Cited by 38 · see citing works
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2025 European LeukemiaNet recommendations for the management of chronic myeloid leukemia
Apperley, J. F., Milojkovic, D., Cross, N. C. P., Hjorth-Hansen, H., Hochhaus, A., Kantarjian, H., Lipton, J. H., Malhotra, H., Niederwieser, D., Radich, J., et al. (2025) · Leukemia · doi:10.1038/s41375-025-02664-w · Cited by 139 · see citing works
Self-citationSystemic mastocytosis in adults: 2023 update on diagnosis, risk stratification and management
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Self-citationAvapritinib versus placebo in indolent systemic mastocytosis
Gotlib, J., Castells, M., Elberink, H. O., Siebenhaar, F., Hartmann, K., Broesby-Olsen, S., George, T. I., Panse, J., Alvarez-Twose, I., Radia, D. H., et al. (2023) · NEJM Evidence · doi:10.1056/evidoa2200339 · Cited by 81 · see citing works
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Novel scheme for defining the clinical implications of TP53 mutations in myeloid neoplasia
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Review and updates on systemic mastocytosis and related entities
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Evidence-based risk stratification of myeloid neoplasms harboring <i>TP53</i> mutations
Shah, M. V., Hung, K., Baranwal, A., Kutyna, M. M., Al-Kali, A., Toop, C., Greipp, P., Brown, A., Shah, S., Khanna, S., et al. (2025) · Blood Advances · doi:10.1182/bloodadvances.2024015238 · Cited by 23 · see citing works
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Disease Spectrum of Anaphylaxis Disorders
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Stahl, M., & Zeidan, A. M. (2026) · Blood · doi:10.1182/blood.2025029727 · Cited by 14 · see citing works
McMurray, J. C., Pacheco, C. S., Schornack, B. J., Sun, X., Brunader, J. A., Scott, A. E., Ariza, J. S., Kou, C.-T. J., Costantino, R. C., Pittman, L. M., et al. (2024) · Blood · doi:10.1182/blood.2023023347 · Cited by 11 · see citing works
Are <i>TP53</i> mutations all alike?
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Pyatilova, P., Bernstein, J. A., Aulenbacher, F., Borges, M. S., Dimitrijević, S., Hoehn, G., Maurer, M., Kolkhir, P., & Siebenhaar, F. (2023) · World Allergy Organization Journal · doi:10.1016/j.waojou.2023.100838 · Cited by 9 · see citing works
Crupi, F., Caroprese, J., & Mannelli, F. (2025) · Frontiers in Allergy · doi:10.3389/falgy.2025.1592001 · Cited by 2 · see citing works
- Authors
- Arber, D. A., Orazi, A., Hasserjian, R. P., Borowitz, M. J., Calvo, K. R., Kvasnicka, H.-M., Wang, S. A., Bagg, A., Barbui, T., Branford, S., Bueso-Ramos, C. E., Cortes, J. E., Dal Cin, P., DiNardo, C. D., Dombret, H., Duncavage, E. J., Ebert, B. L., Estey, E. H., Facchetti, F., Foucar, K., Gangat, N., Gianelli, U., Godley, L. A., Gökbuget, N., Gotlib, J., Hellström-Lindberg, E., Hobbs, G. S., Hoffman, R., Jabbour, E. J., Kiladjian, J.-J., Larson, R. A., Le Beau, M. M., Loh, M. L.-C., Löwenberg, B., Macintyre, E., Malcovati, L., Mullighan, C. G., Niemeyer, C., Odenike, O. M., Ogawa, S., Orfao, A., Papaemmanuil, E., Passamonti, F., Porkka, K., Pui, C.-H., Radich, J. P., Reiter, A., Rozman, M., Rudelius, M., Savona, M. R., Schiffer, C. A., Schmitt-Graeff, A., Shimamura, A., Sierra, J., Stock, W. A., Stone, R. M., Tallman, M. S., Thiele, J., Tien, H.-F., Tzankov, A., Vannucchi, A. M., Vyas, P., Wei, A. H., Weinberg, O. K., Wierzbowska, A., Cazzola, M., Döhner, H., & Tefferi, A.
- Affiliations
- University of Chicago (US)
- Texas Tech University (US)
- Massachusetts General Hospital (US)
- Johns Hopkins University (US)
- National Institutes of Health (US)
- Helios Universitätsklinikum Wuppertal (DE)
- The University of Texas MD Anderson Cancer Center (US)
- University of Pennsylvania (US)
- Ospedale Papa Giovanni XXIII (IT)
- Centre for Cancer Biology (AU)
- 11Georgia Cancer Center, Augusta, GA;
- 12Brigham and Women’s Hospital, Boston, MA;
- Université Paris Cité (FR)
- Washington University in St. Louis (US)
- Dana-Farber Cancer Institute (US)
- University of Washington (US)
- University of Brescia (IT)
- University of New Mexico (US)
- Mayo Clinic (US)
- University of Milan (IT)
- Goethe University Frankfurt (DE)
- Stanford Medicine (US)
- 23Karolinska Institutet, Solna, Sweden;
- Mount Sinai Hospital (US)
- 1University of Chicago, Chicago, IL;
- Seattle Children's Hospital (US)
- Erasmus MC (NL)
- University of Pavia (IT)
- St. Jude Children's Research Hospital (US)
- University of Freiburg (DE)
- Kyoto University (JP)
- 32University of Salamanca, Salamanca, Spain;
- Memorial Sloan Kettering Cancer Center (US)
- Helsinki University Hospital (FI)
- Fred Hutch Cancer Center (US)
- University Medical Centre Mannheim (DE)
- 37University of Barcelona, Barcelona, Spain;
- Ludwig-Maximilians-Universität München (DE)
- Vanderbilt University (US)
- The Barbara Ann Karmanos Cancer Institute (US)
- Hospital de Sant Pau (ES)
- Lurie Children's Hospital (US)
- University of Cologne (DE)
- National Taiwan University Hospital (TW)
- 47Medical University Basel, Basel, Switzerland;
- University of Florence (IT)
- University of Oxford (GB)
- The Royal Melbourne Hospital (AU)
- The University of Texas Southwestern Medical Center (US)
- Medical University of Lodz (PL)
- University Hospital Ulm (DE)
- ORCID
- Arber, Daniel A.
- Orazi, Attilio
- Hasserjian, Robert P.
- Calvo, Katherine R.
- Kvasnicka, Hans-Michael
- Wang, Sa A.
- Bagg, Adam
- Barbui, Tiziano
- Branford, Susan
- Bueso-Ramos, Carlos E.
- Cortes, Jorge E.
- DiNardo, Courtney D.
- Dombret, Hervé
- Ebert, Benjamin L.
- Estey, Elihu H.
- Facchetti, Fabio
- Foucar, Kathryn
- Gangat, Naseema
- Gianelli, Umberto
- Godley, Lucy A.
- Gökbuget, Nicola
- Gotlib, Jason
- Hobbs, Gabriela S.
- Hoffman, Ronald
- Jabbour, Elias J.
- Kiladjian, Jean-Jacques
- Larson, Richard A.
- Loh, Mignon L.-C.
- Löwenberg, Bob
- Macintyre, Elizabeth
- Malcovati, Luca
- Mullighan, Charles G.
- Niemeyer, Charlotte
- Odenike, Olatoyosi M.
- Ogawa, Seishi
- Papaemmanuil, Elli
- Passamonti, Francesco
- Porkka, Kimmo
- Pui, Ching-Hon
- Radich, Jerald P.
- Reiter, Andreas
- Rudelius, Martina
- Savona, Michael R.
- Schiffer, Charles A.
- Schmitt-Graeff, Annette
- Shimamura, Akiko
- Sierra, Jorge
- Stock, Wendy A.
- Stone, Richard M.
- Tallman, Martin S.
- Tien, Hwei-Fang
- Tzankov, Alexandar
- Vannucchi, Alessandro M.
- Vyas, Paresh
- Wei, Andrew H.
- Weinberg, Olga K.
- Wierzbowska, Agnieszka
- Cazzola, Mario
- Döhner, Hartmut
- Tefferi, Ayalew
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2022-09-15
- Volume
- 140
- Issue
- 11
- Pages
- 1200-1228
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- PMC
- PMC9479031
- OpenAlex
- W4283716970
- Cited by
- 2,945
- References
- 261
- Citations by year
- 2026: 665 · 2025: 791 · 2024: 752 · 2023: 625 · 2022: 107
- FWCI
- 414.74
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Blood (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Kölner Universitäts PublikationsServer (Universität zu Köln) (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (publishedVersion, other-oa)
- Digital Commons-TMC (Texas Medical Center) (submittedVersion)
- UNM’s Digital Repository (University of New Mexico) (submittedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
- Adelaide Research & Scholarship (AR&S) (University of Adelaide) (submittedVersion)
- Florence Research (University of Florence) (submittedVersion)
- Funders
- National Institute for Health and Care Research (NF-SI-0617-10056)
- Medical Research Council (MC_PC_19049, MC_UU_00029/8, MC_UU_00016/11, MR/L008963/1)
- National Cancer Institute (P30CA008748, P01CA066996)
- Topics
- Health Sciences › Medicine › Hematology › Acute Myeloid Leukemia Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinoids in leukemia and cellular processes
- Health Sciences › Medicine › Emergency Medicine › Hematological disorders and diagnostics
- Keywords
- myeloid neoplasms, acute leukemia, International Consensus Classification, disease classification, diagnostic criteria, disease entity, morphologic features, clinical features, genomic data, outcome prediction, hematologic malignancies
- MeSH terms
- Acute Disease, Humans, Leukemia, Myeloproliferative Disorders, World Health Organization, Hematologic Neoplasms, Genomics, Consensus
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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