AUTHOR=Fontana Diletta , Gambacorti-Passerini Carlo , Piazza Rocco TITLE=Molecular Pathogenesis of BCR-ABL-Negative Atypical Chronic Myeloid Leukemia JOURNAL=Frontiers in Oncology VOLUME=11 YEAR=2021 URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2021.756348 DOI=10.3389/fonc.2021.756348 ISSN=2234-943X ABSTRACT=

Atypical chronic myeloid leukemia is a rare disease whose pathogenesis has long been debated. It currently belongs to the group of myelodysplastic/myeloproliferative disorders. In this review, an overview on the current knowledge about diagnosis, prognosis, and genetics is presented, with a major focus on the recent molecular findings. We describe here the molecular pathogenesis of the disease, focusing on the mechanisms of action of the main mutations as well as on gene expression profiling. We also present the treatment options focusing on emerging targeted therapies.