{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T08:06:22Z","timestamp":1793088382680,"version":"4.5.0"},"reference-count":20,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T00:00:00Z","timestamp":1759276800000},"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:p>In recent years, acute myeloid leukemia with plasmacytoid dendritic cell expansion (pDC-AML) has been recognized as a rare provisional subtype of AML, comprising approximately 3\u20135% of all reported cases and associated with a poorer clinical outcome compared with non\u2013pDC-AML. Both <jats:italic>RUNX1<\/jats:italic> mutations and rare rearrangements can lead to either complete loss or dominant-negative inhibition of RUNX1 function in pDC-AML, which may play a pivotal role in the aberrant expansion or malignant transformation of plasmacytoid dendritic cells (pDCs). To date, only two cases of pDC-AML with rare <jats:italic>RUNX1<\/jats:italic> rearrangements have been reported. Herein, we reported a rare <jats:italic>RUNX1<\/jats:italic> rearrangement resulting from t(8;21)(p12;q22) in a patient with pDC-AML, leading to the truncated RUNX1 that exhibit structural and functional similarities to RUNX1A and may act as a dominant-inhibitor of wild-type RUNX1. Given the poor prognosis associated with this subtype, CD123-targeted therapy, such as tagraxofusp-erzs, alone or in combination with agents like azacitidine and venetoclax, may represent a rational therapeutic approach. To our knowledge, this represents the third case report of <jats:italic>RUNX1<\/jats:italic> rearrangement in pDC-AML and may provide valuable insights for future research.<\/jats:p>","DOI":"10.3389\/fonc.2025.1672416","type":"journal-article","created":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T04:17:37Z","timestamp":1759292257000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case Report: A rare RUNX1 rearrangement resulting from t(8;21)(p12;q22) in acute myeloid leukemia with plasmacytoid dendritic cell expansion"],"prefix":"10.3389","volume":"15","author":[{"given":"Jinying","family":"Gong","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jialong","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisha","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiru","family":"Wei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xue","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junyan","family":"Zou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuan","family":"Feng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guoqing","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing","family":"Han","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,10,1]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020007897","article-title":"Plasmacytoid 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