ArticleActa haematologica2016
Molecular Characteristics and Clinical Significance of 12 Fusion Genes in Acute Promyelocytic Leukemia: A Systematic Review.
Article in Acta haematologica, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 18 citations in OpenAlex.
- The appearance of "faggot Auer rods" in blasts of non-M3 acute myeloid leukemia.Frontiers in oncology · 2026Article
- Identification of a novel fusion gene, RARA::ANKRD34C, in acute promyelocytic leukemia.Annals of hematology · 2024Article
- ZBTB16-RARα-Positive Atypical Promyelocytic Leukemia: A Case Report.Medicina (Kaunas, Lithuania) · 2022Article
- Arsenic trioxide induces proteasome dependent TBLR1-RARα degradation to improve leukemia eradication through cell differentiation enhancement.Journal of Cancer · 2022Article
- Review
- Clinical and molecular features of acute promyelocytic leukemia with variant retinoid acid receptor fusions.Haematologica · 2019Article
- Serendipity: decitabine monotherapy induced complete molecular response in a 77-year-old patient with acute promyelocytic leukemia.Haematologica · 2019Article
- RNF8 is responsible for ATRA resistance in variant acute promyelocytic leukemia with GTF2I/RARA fusion, and inhibition of the ubiquitin-proteasome pathway contributes to the reversion of ATRA resistance.Cancer cell international · 2019Article
- A rare case of acute promyelocytic leukemia with IRF2BP2-RARA fusion; and literature review.OncoTargets and therapy · 2019Article
- [Chinese guidelines for diagnosis and treatment of acute promyelocytic leukemia (2018)].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2018Article
- Analysis of the oligomeric states of nucleophosmin using size exclusion chromatography.Scientific reports · 2018Article
- Acute Promyelocytic Leukemia in Children: A Single Centre Experience from Turkey.Mediterranean journal of hematology and infectious diseases · 2018Article
- Molecular Profiling: A Case ofCase reports in hematology · 2017Article
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Acute promyelocytic leukemia (APL) is characterized by the generation of the promyelocytic leukemia-retinoic acid (RA) receptor α (PML-RARα) fusion gene. PML-RARα is the central leukemia-initiating event in APL and is directly targeted by all-trans-RA (ATRA) as well as arsenic. In classic APL harboring PML-RARα transcripts, more than 90% of patients can achieve complete remission when treated with ATRA combined with arsenic trioxide chemotherapy. In the last 20 years, more than 10 variant fusion genes have been found and identified in APL patients. These variant APL cases present different clinical phenotypes and treatment outcomes. All variant APL cases show a similar breakpoint within the RARα gene, whereas its partner genes are variable. These fusion proteins have the ability to repress rather than activate retinoic targets. These chimeric proteins also possess different molecular characteristics, thereby resulting in variable sensitivities to ATRA and clinical outcomes. In this review, we comprehensively analyze various rearrangements in variant APL cases that have been reported in the literature as well as the molecular characteristics and functions of the fusion proteins derived from different RARα partner genes and their clinical implications.
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