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Review
. 2024 Dec 16;16(24):4192.
doi: 10.3390/cancers16244192.

Acute Promyelocytic Leukemia-like AML: Genetic Perspective and Clinical Implications

Luca Guarnera  1 , Emiliano Fabiani  2   3 , Giulia Falconi  2   4 , Giorgia Silvestrini  1 , Maria Luigia Catanoso  2 , Mariadomenica Divona  4 , Maria Teresa Voso  2   5
Affiliations
Review

Acute Promyelocytic Leukemia-like AML: Genetic Perspective and Clinical Implications

Luca Guarnera et al. Cancers (Basel). .

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.

Keywords: APL; APL-like AMLs; PLZF::RARA; RARA; RARB; RARG; acute promyelocytic leukemia; atypical rearrangements.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Overview of rearrangement partners of RAR genes. The color of partner genes matches the color of RAR genes with whom rearrangements were described. Some genes were described as partners of more than one component of RAR family and are shown in the overlapping areas between circles.
Figure 2
Figure 2
Histogram showing the number of reports on new fusion partners described in APL-like AMLs over time. AML: acute myeloid leukemia; APL: acute promyelocytic leukemia, No: number.
See this image and copyright information in PMC

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