2020
DOI: 10.3390/cancers12030624
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Acute Promyelocytic Leukemia: A Constellation of Molecular Events around a Single PML-RARA Fusion Gene

Abstract: Although acute promyelocytic leukemia (APL) is one of the most characterized forms of acute myeloid leukemia (AML), the molecular mechanisms involved in the development and progression of this disease are still a matter of study. APL is defined by the PML-RARA rearrangement as a consequence of the translocation t(15;17)(q24;q21). However, this abnormality alone is not able to trigger the whole leukemic phenotype and secondary cooperating events might contribute to APL pathogenesis. Additional somatic mutations… Show more

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Cited by 107 publications
(111 citation statements)
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References 164 publications
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“…Since the discovery of PML-RARA, more than a dozen diverse translocations involving RARA have been found in rare leukemia patients, often with typical morphological features of APL [57][58][59]. More recently, very rare fusions involving other retinoic acid receptors have also been described (Table 1, Figure 2) [60].…”
Section: Novel Retinoic Acid Receptors Fusions In Aplmentioning
confidence: 99%
“…Since the discovery of PML-RARA, more than a dozen diverse translocations involving RARA have been found in rare leukemia patients, often with typical morphological features of APL [57][58][59]. More recently, very rare fusions involving other retinoic acid receptors have also been described (Table 1, Figure 2) [60].…”
Section: Novel Retinoic Acid Receptors Fusions In Aplmentioning
confidence: 99%
“…In particular, the breakpoint of the RARA gene is located in its second intron, while three different breakpoints of the PML gene (intron 6, exon 6, and intron 3) identify the most common breakpoint cluster regions (bcr-1, -2, and -3, respectively), corresponding to different PML-RARA isoforms [ 12 ]. While bcr-1 (also known as “long” or “L” isoform) and bcr-3 (also known as “short” or “S” isoform) account for the majority of APL cases (90–95%), bcr-2 represents a more rare variant (also called “V”) [ 13 ] and no particular differences (apart from those related to ethnicity) are known as to the different frequency of these isoforms between children and adults [ 14 , 15 ].…”
Section: Epidemiology and Biology Of Acute Promyelocytic Leukemiamentioning
confidence: 99%
“…More detailed analyses have shown the relationship between a poor outcome and several characteristics, including older age, chromosomal abnormalities other than t (15;17), phenotypic features, FLT3 mutations and presence of the PML-RARA isoform [ 13 , 14 , 15 , 16 , 17 ]. However, these observations have not received approval to amend the standard therapy for APL [ 18 , 19 , 20 ].…”
Section: Introductionmentioning
confidence: 99%