{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T04:46:54Z","timestamp":1776746814438,"version":"3.51.2"},"reference-count":16,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T00:00:00Z","timestamp":1776729600000},"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":"\n Background<\/jats:title>\n \n KMT2A::AFF1<\/jats:italic>\n -rearranged ALL is a high-risk subtype with an aggressive course. However, the simultaneous occurrence of catastrophic intracranial hemorrhage (ICH) and spontaneous tumor lysis syndrome (TLS) as the initial presentation is rare in adult patients and remains poorly characterized in the literature.\n <\/jats:p>\n <\/jats:sec>\n \n Case Presentation<\/jats:title>\n \n A previously healthy 19-year-old Chinese male was admitted with sudden-onset drowsiness and impaired consciousness. Initial computed tomography revealed multiple intracranial hemorrhages with cerebral swelling. Laboratory findings were notable for extreme hyperleukocytosis (554.66 \u00d7 10\n 9<\/jats:sup>\n \/L) with 85% blasts (some with cup-like morphology), severe thrombocytopenia, and biochemical evidence of TLS. Despite emergency neurosurgical intervention and intensive supportive care, his clinical course was marked by the rapid progression of TLS and acute kidney injury, culminating in fatal cardiac arrest within 24 hours of admission. Postmortem flow cytometry and genetic analysis confirmed the diagnosis of pro-B ALL positive for the\n KMT2A::AFF1<\/jats:italic>\n fusion gene.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n \n This case illustrates an exceptionally aggressive manifestation of adult\n KMT2A<\/jats:italic>\n -rearranged ALL, characterized by the concurrent development of ICH and spontaneous TLS prior to the initiation of any leukemia-specific therapy. It underscores the critical importance of promptly recognizing this hyperacute syndrome in young patients presenting with unexplained neurological symptoms and profound hematologic abnormalities. Early multidisciplinary management and awareness of high-risk genetic profiles are essential, although the prognosis in such scenarios remains exceedingly poor.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.3389\/fonc.2026.1716717","type":"journal-article","created":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T04:22:58Z","timestamp":1776745378000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Concurrent intracranial hemorrhage and spontaneous tumor Lysis syndrome as the initial presentation of KMT2A::AFF1-rearranged adult ALL: a case report"],"prefix":"10.3389","volume":"16","author":[{"given":"Yuyi","family":"Lai","sequence":"first","affiliation":[{"name":"Department of Clinical Laboratory, The First People\u2019s Hospital of Nanning","place":["Nanning, China"]},{"name":"Department of Clinical Laboratory, The Fifth Affiliated Hospital of Guangxi Medical University","place":["Nanning, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ligang","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Clinical Laboratory, The First People\u2019s Hospital of Nanning","place":["Nanning, China"]},{"name":"Department of Clinical Laboratory, The Fifth Affiliated Hospital of Guangxi Medical University","place":["Nanning, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Min","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Clinical Laboratory, The First People\u2019s Hospital of Nanning","place":["Nanning, China"]},{"name":"Department of Clinical Laboratory, The Fifth Affiliated Hospital of Guangxi Medical University","place":["Nanning, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rongguo","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Clinical Laboratory, The First People\u2019s Hospital of Nanning","place":["Nanning, China"]},{"name":"Department of Clinical Laboratory, The Fifth Affiliated Hospital of Guangxi Medical University","place":["Nanning, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,4,21]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1038\/s41582-024-01035-w","article-title":"Intracerebral hemorrhage \u2014 mechanisms, diagnosis and prospects for treatment and prevention","volume":"20","author":"Seiffge","year":"2024","journal-title":"Nat Rev Neurol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1159\/000341805","article-title":"Leukocyte depletion by therapeutic leukocytapheresis in patients with leukemia","volume":"39","author":"H\u00f6lig","year":"2012","journal-title":"Transfus Med Hemotherapy"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V97.7.2121","article-title":"Endothelial cell activation by myeloblasts: molecular mechanisms of leukostasis and leukemic cell 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Hematol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra0904569","article-title":"The tumor lysis syndrome","volume":"364","author":"Howard","year":"2011","journal-title":"N Engl J Med"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2024028019","article-title":"Enhancer heterogeneity in acute lymphoblastic leukemia drives differential gene expression in patients","volume":"146","author":"Smith","year":"2025","journal-title":"Blood"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2021004580","article-title":"Outcomes of acute lymphoblastic leukemia with KMT2A (MLL) rearrangement: the MD Anderson experience","volume":"5","author":"Richard-Carpentier","year":"2021","journal-title":"Blood Adv"},{"key":"B10","doi-asserted-by":"publisher","first-page":"1469","DOI":"10.1182\/blood-2019-121977","article-title":"Prognostic impact of t(4;11)(q21;q23)\/KMT2A-AFF1-positivity in 926 BCR-ABL1-negative B-lineage acute lymphoblastic leukemia patients treated in GIMEMA clinical trials since 1996","volume":"134","author":"Piciocchi","year":"2019","journal-title":"Blood"},{"key":"B11","unstructured":"Recent Developments and Evolving Therapeutic Strategies in KMT2A-Rearranged Acute Leukemia.\n \n \n \n Yin\n L\n \n \n Wan\n L\n \n \n Zhang\n Y\n \n \n Hua\n S\n \n \n Shao\n X\n \n \n 10.1002\/cam4.70326\n 39428967\n Cancer Medicine\n 3\n 2024"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1007\/s12308-019-00355-x","article-title":"Characterization of a cryptic KMT2A\/AFF1 gene fusion by mate-pair sequencing (MPseq) in a young adult with newly diagnosed B-lymphoblastic leukemia","volume":"12","author":"Peterson","year":"2019","journal-title":"Journal of Hematopathology"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.3390\/biomedicines11030821","article-title":"Updates in KMT2A gene rearrangement in pediatric acute lymphoblastic 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Hematol"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1716717\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T04:22:59Z","timestamp":1776745379000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1716717\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,21]]},"references-count":16,"alternative-id":["10.3389\/fonc.2026.1716717"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2026.1716717","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,21]]},"article-number":"1716717"}}