{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,1]],"date-time":"2026-11-01T21:36:26Z","timestamp":1793568986197,"version":"4.5.0"},"reference-count":60,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,11,17]],"date-time":"2021-11-17T00:00:00Z","timestamp":1637107200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>Atypical chronic myelogenous leukemia (aCML), <jats:italic>BCR\/ABL1<\/jats:italic> negative is a rare myelodysplastic\/myeloproliferative neoplasm, usually manifested with hyperleukocytosis without monocytosis or basophilia, organomegaly, and marked dysgranulopoiesis. In this review, we will discuss the classification and diagnostic criteria of aCML, as these have been formulated during the past 30 years, with a focus on the recent advances in the molecular characterization of the disease. Although this entity does not have a definitive molecular profile, its molecular characterization has contributed to a better understanding and more accurate classification and diagnosis of aCML. At the same time, it has facilitated the identification of adverse prognostic factors and the stratification of patients according to their risk for leukemic transformation. What is more, the molecular characterization of the disease has expanded our therapeutic choices, thoroughly presented and analyzed in this review article.<\/jats:p>","DOI":"10.3389\/fonc.2021.722507","type":"journal-article","created":{"date-parts":[[2021,11,17]],"date-time":"2021-11-17T07:05:11Z","timestamp":1637132711000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches"],"prefix":"10.3389","volume":"11","author":[{"given":"Panagiotis T.","family":"Diamantopoulos","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nora-Athina","family":"Viniou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2021,11,17]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-03-643544","article-title":"The 2016 Revision to the World Health Organization Classification of Myeloid Neoplasms and Acute Leukemia","volume":"127","author":"Arber","year":"2016","journal-title":"Blood"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-03-209262","article-title":"The 2008 Revision of the World Health Organization (WHO) Classification of Myeloid Neoplasms and Acute Leukemia: Rationale and Important Changes","volume":"114","author":"Vardiman","year":"2009","journal-title":"Blood"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.5114\/wo.2018.74388","article-title":"Atypical Chronic Myeloid Leukaemia - A Rare Subtype of Myelodysplastic\/Myeloproliferative Neoplasm","volume":"22","author":"Drozd-Soko\u0142owska","year":"2018","journal-title":"Contemp Oncol (Pozn)"},{"key":"B4","first-page":"3","article-title":"[Does Ph\u2019-Negative Chronic Myeloleukemia Exist]","volume":"34","author":"Iavorkovski\u012d","year":"1989","journal-title":". 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