{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T04:55:55Z","timestamp":1789016155670,"version":"build-2803163510"},"reference-count":37,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,1,4]],"date-time":"2023-01-04T00:00:00Z","timestamp":1672790400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Disease Overview<\/jats:title><jats:p>Atypical chronic myeloid leukemia (aCML) and myelodysplastic\/myeloproliferative (MDS\/MPN) neoplasms, not otherwise specified (NOS), are MDS\/MPN overlap neoplasms characterized by leukocytosis, in the absence of monocytosis and eosinophilia, with &lt;20% blasts in the blood and bone marrow.<\/jats:p><\/jats:sec><jats:sec><jats:title>Diagnosis<\/jats:title><jats:p>aCML, previously known as aCML, <jats:italic>BCR::ABL1<\/jats:italic> negative, was renamed as aCML by the ICC classification, and as MDS\/MPN with neutrophilia by the 5th edition of the WHO classification. This entity is characterized by dysplastic neutrophilia with immature myeloid cells comprising \u226510% of the white blood cell count, with prominent dysgranulopoiesis. MDS\/MPN\u2010NOS consists of MDS\/MPN overlap neoplasms not meeting criteria for defined categories such as chronic myelomonocytic leukemia (CMML), MDS\/MPN\u2010ring sideroblasts\u2010thrombocytosis (MDS\/MPN\u2010RS\u2010T), and aCML.<\/jats:p><\/jats:sec><jats:sec><jats:title>Mutations and Karyotype<\/jats:title><jats:p>Cytogenetic abnormalities are seen in 40\u201350% of patients in both categories. In aCML, somatic mutations commonly encountered include <jats:italic>ASXL1<\/jats:italic>, <jats:italic>SETBP1<\/jats:italic>, <jats:italic>ETNK1<\/jats:italic>, and <jats:italic>EZH2<\/jats:italic> whereas MDS\/MPN\u2010NOS can be further stratified by mutational profiles into CMML\u2010like, MDS\/MPN\u2010RS\u2010T\u2010like, aCML\u2010like, <jats:italic>TP35<\/jats:italic>\u2010mutated, and \u201cothers\u201d, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk Stratification<\/jats:title><jats:p>The Mayo Clinic aCML model stratifies patients based on age &gt;67\u2009years, hemoglobin &lt;10\u00a0g\/dl, and the presence of <jats:italic>TET2<\/jats:italic> mutations into low\u2010risk (0\u20131 points) and high\u2010risk (&gt;2 points) groups, with median survivals of 18 and 7\u00a0months, respectively. MDS\/MPN\u2010NOS patients have traditionally been risk stratified using MDS risk models such as IPSS and IPSS\u2010R.<\/jats:p><\/jats:sec><jats:sec><jats:title>Treatment<\/jats:title><jats:p>Leukocytosis and anemia are managed like lower risk MPN and MDS. DNMT inhibitors have been used in both entities with suboptimal response rates. Allogeneic stem cell transplant remains the only curative strategy but is associated with high morbidity and mortality.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.26828","type":"journal-article","created":{"date-parts":[[2023,1,5]],"date-time":"2023-01-05T07:45:52Z","timestamp":1672904752000},"page":"681-689","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Atypical chronic myeloid leukemia and myelodysplastic\/myeloproliferative neoplasm, not otherwise specified: 2023 update on diagnosis, risk stratification, and management"],"prefix":"10.1002","volume":"98","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6998-662X","authenticated-orcid":false,"given":"Mrinal M.","family":"Patnaik","sequence":"first","affiliation":[{"name":"Division of Hematology Department of Internal Medicine, Mayo Clinic  Rochester Minnesota 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