ABSTRACT
Atypical chronic myeloid leukemia (aCML) is a rare hematologic malignancy with both bcr-abl-negative and the absence of the Philadelphia chromosome, which is completely different from chronic myeloid leukemia (CML). According to the clinical characteristics and the main hematological characteristics, it is classified as myelodysplastic/myeloproliferative neoplasms. Due to the low incidence and limited understanding of aCML, it can result in the clinical missed diagnosis or misdiagnosis, and the short survival time of patients. In order to further improve the diagnostic rate and survival time, this paper reviews the current diagnostic criteria, differential diagnosis, related gene mutations, prognosis evaluation and clinical treatments of aCML.