2022
DOI: 10.1002/ajh.26751
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The international consensus classification of myeloid neoplasms and acute Leukemias: myeloproliferative neoplasms

Abstract: A group of international experts, including hematopathologists, oncologists, and geneticists were recently summoned (September 2021, Chicago, IL, USA) to update the 2016/17 World Health Organization classification system for hematopoietic tumors. After careful deliberation, the group introduced the new International Consensus Classification (ICC) for Myeloid Neoplasms and Acute Leukemias. This current in‐depth review focuses on the ICC‐2022 category of JAK2 mutation‐prevalent myeloproliferative neoplasms (MPNs… Show more

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Cited by 49 publications
(54 citation statements)
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“…Polycythemia vera (PV) is one of four JAK2 mutation‐prevalent myeloproliferative neoplasms (MPN), which also include essential thrombocythemia (ET), primary myelofibrosis (PMF), and MPN, unclassifiable (MPN‐U) 1–3 . In addition, about 15% of patients with PV or ET develop a PMF‐like phenotype, over time, referred to as post‐ET or post‐PV myelofibrosis 4 .…”
Section: Disease Classification and Incidencementioning
confidence: 99%
“…Polycythemia vera (PV) is one of four JAK2 mutation‐prevalent myeloproliferative neoplasms (MPN), which also include essential thrombocythemia (ET), primary myelofibrosis (PMF), and MPN, unclassifiable (MPN‐U) 1–3 . In addition, about 15% of patients with PV or ET develop a PMF‐like phenotype, over time, referred to as post‐ET or post‐PV myelofibrosis 4 .…”
Section: Disease Classification and Incidencementioning
confidence: 99%
“…2 Morphologically, all three MPN variants display variable degrees of trilineage myeloproliferation associated with bone marrow stromal reaction that is most intense in MF, where abnormal megakaryocyte proliferation is often accompanied by bone marrow fibrosis, ineffective erythropoiesis (clinically apparent as anemia), aberrant cytokine expression (clinically apparent as constitutional symptoms and cachexia), and extramedullary hematopoiesis (EMH; clinically apparent as hepatosplenomegaly). 3 The three MPN variants also differ in disease course, survival outcome, and risk of progression into blast-phase disease (MPN-BP); reported median survivals for PMF, PV and ET were 4.4, 15, and 18 years, respectively, with corresponding leukemic transformation rates of 9.3%, 3.9%, and 2.6%. 4 Patients with MF are subject to not only premature death 4 but also poor quality-of-life (QoL).…”
Section: Introductionmentioning
confidence: 99%
“…The ICC MPN subcommittee retained most of the structure and content from the 2016/17 WHO classification (revised 4th edition), in regard to the JAK2 mutation‐prevalent MPNs 2,8 . In CML, ICC recognizes three disease phases: chronic (CP), accelerated (AP), and blast (BP) phase 1 .…”
Section: Changes In Mds/mpn and Mpn Disease Subcategoriesmentioning
confidence: 99%
“…The most recent publication from the ICC‐2022 provides both basic and advanced information on molecular biology and elaborates on modern genomic diagnostics and risk stratification, as it relates to ICC‐assigned disease entities 7 . In the current editorial, which accompanies the first of a series of ICC‐2022 review articles that are slated to be published in the American Journal of Hematology , we highlight some of the changes in ICC‐2022, 8 compared to the 2016/17 WHO classification (revised 4th edition) 2 …”
mentioning
confidence: 99%