Classic and Variants APLs, as Viewed from a Therapy Response.
ATO
ATRA
PML
leukemia
retinoic acid signaling
targeted therapy
Journal
Cancers
ISSN: 2072-6694
Titre abrégé: Cancers (Basel)
Pays: Switzerland
ID NLM: 101526829
Informations de publication
Date de publication:
14 Apr 2020
14 Apr 2020
Historique:
received:
24
03
2020
revised:
09
04
2020
accepted:
09
04
2020
entrez:
17
4
2020
pubmed:
17
4
2020
medline:
17
4
2020
Statut:
epublish
Résumé
Most acute promyelocytic leukemia (APL) are caused by PML-RARA, a translocation-driven fusion oncoprotein discovered three decades ago. Over the years, several other types of rare X-RARA fusions have been described, while recently, oncogenic fusion proteins involving other retinoic acid receptors (RARB or RARG) have been associated to very rare cases of acute promyelocytic leukemia. PML-RARA driven pathogenesis and the molecular basis for therapy response have been the focus of many studies, which have now converged into an integrated physio-pathological model. The latter is well supported by clinical and molecular studies on patients, making APL one of the rare hematological disorder cured by targeted therapies. Here we review recent data on APL-like diseases not driven by the PML-RARA fusion and discuss these in view of current understanding of "classic" APL pathogenesis and therapy response.
Identifiants
pubmed: 32295268
pii: cancers12040967
doi: 10.3390/cancers12040967
pmc: PMC7226009
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
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