{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,14]],"date-time":"2026-01-14T20:02:19Z","timestamp":1768420939982,"version":"3.49.0"},"reference-count":26,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,2,21]],"date-time":"2024-02-21T00:00:00Z","timestamp":1708473600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Acute promyelocytic leukemia (APL) with <jats:italic>PML\/RAR\u03b1<\/jats:italic> fusion gene is a distinct variant of acute myeloid leukemia. According to the different break sites of the <jats:italic>PML<\/jats:italic> gene, there are three transcripts: Long (bcr1), Variant (bcr2) and Short (bcr3).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively analyzed 82 APL cases with <jats:italic>PML-RAR\u03b1<\/jats:italic> short isoform.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 384 patients with APL were seen, of which 85(22.14%) had <jats:italic>PML\/RAR\u03b1<\/jats:italic> short isoform (bcr3) and 82 met the inclusion criteria. The median age was 33.5 years (range, 2-72 years). The incidences of hemorrhage in the intermediate- and high-risk group were higher, but only the incidence between medium and low risk differed statistically (<jats:italic>P=0.006)<\/jats:italic>, and the incidences of fever, fatigue, splenomegaly, and lymph node enlargement and differentiation syndrome (DS<jats:italic>)<\/jats:italic> in those groups were not statistically significant (<jats:italic>P<\/jats:italic>&amp;gt;0.05). <jats:italic>FLT3<\/jats:italic> gene mutation rate and the mortality rate of the high-risk group were significantly higher than that of other groups (<jats:italic>P<\/jats:italic>=0.040 and <jats:italic>P<\/jats:italic>=0.004, <jats:italic>P=<\/jats:italic>0.041 and <jats:italic>P<\/jats:italic>=0.037, respectively). The mortality rate was lowest (4.26%) in the group treated with ATRA combined with arsenic and anthracycline. The 3-year OS and the 3-year DFS of the low and intermediate-risk group were better (<jats:italic>P<\/jats:italic>=0.019 and <jats:italic>P<\/jats:italic>=0.017, respectively).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>ATRA combined with arsenic and anthracycline had significant impact on outcomes in APL with <jats:italic>PML-RAR\u03b1<\/jats:italic> short isoform.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fonc.2024.1342671","type":"journal-article","created":{"date-parts":[[2024,2,21]],"date-time":"2024-02-21T05:18:22Z","timestamp":1708492702000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Clinical analysis of 82 cases of acute promyelocytic leukemia with PML-RAR\u03b1 short isoform in children and adults"],"prefix":"10.3389","volume":"14","author":[{"given":"Qiaolin","family":"Huang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yicheng","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miao","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,2,21]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.3969\/j.issn.1672-2353.2003.04.014","article-title":"Clinical study of PML-RAR\u03b1 fusion gene in acute promyelocytic leukemia","author":"Jiang","year":"2003","journal-title":"J Pract Clin Med"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.11.4840","article-title":"Expression pattern of the RAR alpha-PML fusion gene in acute promyelocytic 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