Abstract
Acute promyelocytic leukemia (APL) is a rare type of AML, characterized by the t(15;17) translocation and accounting for 8-15% of cases. The introduction of target therapies, such as all-trans retinoic acid (ATRA) and arsenic trioxide (ATO), radically changed the management of APL, making it the most curable AML subtype. However, a small percentage (estimated to be 2%) of AML presenting with APL-like morphology and/or immunophenotype lacks t(15;17). This rare APL-like AML group, whose first case was described in the early 1990s, now includes over 40 entities. These diseases present great heterogeneity in terms of genetic lesions, clinical presentation, sensitivity to targeted agents and chemotherapy, and prognosis. Furthermore, the diagnosis is very challenging. Thus, in this paper, we aim to comprehensively review the literature reports and studies addressing APL-like entities, investigate the biological mechanisms of leukemogenesis, evaluate the clinical characteristics, and discuss future lines of research and possible clinical approaches.