Atypical chronic myelogenous leukemia (aCML), BCR/ABL1 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