{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:36:56Z","timestamp":1787027816773,"version":"build-2736575974"},"reference-count":50,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2024,1,28]],"date-time":"2024-01-28T00:00:00Z","timestamp":1706400000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"NIGMS\/NIH","award":["R35GM151109"],"award-info":[{"award-number":["R35GM151109"]}]},{"name":"NIGMS\/NIH","award":["K08CA230319"],"award-info":[{"award-number":["K08CA230319"]}]},{"name":"NCI\/NIH","award":["R35GM151109"],"award-info":[{"award-number":["R35GM151109"]}]},{"name":"NCI\/NIH","award":["K08CA230319"],"award-info":[{"award-number":["K08CA230319"]}]},{"name":"Doris Duke Charitable Foundation","award":["R35GM151109"],"award-info":[{"award-number":["R35GM151109"]}]},{"name":"Doris Duke Charitable Foundation","award":["K08CA230319"],"award-info":[{"award-number":["K08CA230319"]}]},{"name":"Edward P. Evans Foundation","award":["R35GM151109"],"award-info":[{"award-number":["R35GM151109"]}]},{"name":"Edward P. Evans Foundation","award":["K08CA230319"],"award-info":[{"award-number":["K08CA230319"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy with poor outcomes. The World Health Organization (WHO) redefined BDCN as a distinct disease entity in 2016. BPDCN arises from plasmacytoid dendritic cells, manifesting primarily in the skin, bone marrow, and lymph nodes, occasionally involving the central nervous system (CNS). This presents challenges in diagnosis and treatment, with CNS involvement often overlooked in standard diagnostic workups due to BPDCN\u2019s rarity and patients often being neurologically asymptomatic at diagnosis. CNS involvement typically emerges during relapse, yet clinical trials often exclude such cases, limiting our understanding of its development and treatment. Treatment options for CNS involvement include intrathecal (IT) chemotherapies like methotrexate and cytarabine, often in combination with systemic agents. Tagraxofusp and traditional regimens for acute myeloid leukemia show limited success at preventing CNS relapse, prompting exploration of combined therapies like hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (HyperCVAD) with venetoclax and adding IT chemotherapy to other backbones. Ongoing clinical trials investigating emerging therapies offer hope despite limited focus on CNS implications. Trials incorporating CNS-involved patients aim to pioneer novel treatment approaches, potentially reshaping BPDCN management. Understanding CNS involvement\u2019s complexities in BPDCN remains crucial for tailored treatments and better patient outcomes.<\/jats:p>","DOI":"10.3390\/cells13030243","type":"journal-article","created":{"date-parts":[[2024,1,29]],"date-time":"2024-01-29T08:54:03Z","timestamp":1706518443000},"page":"243","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Blastic Plasmacytoid Dendritic Cell Neoplasm: A Comprehensive Review of the Disease, Central Nervous System Presentations, and Treatment Strategies"],"prefix":"10.3390","volume":"13","author":[{"given":"Shefali","family":"Mehra","sequence":"first","affiliation":[{"name":"Sylvester Comprehensive Cancer Center, Miller School of Medicine, University of Miami, Miami, FL 33136, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4407-6325","authenticated-orcid":false,"given":"Justin","family":"Taylor","sequence":"additional","affiliation":[{"name":"Sylvester Comprehensive Cancer Center, Miller School of Medicine, University of Miami, Miami, FL 33136, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,1,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1111\/ejh.13959","article-title":"Incidence, prognostic factors, and survival outcomes in patients with blastic plasmacytoid dendritic cell neoplasm: A retrospective study in the Surveillance, Epidemiology, and End Results database","volume":"110","author":"Lin","year":"2023","journal-title":"Eur. 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