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BPDCN cells characteristically express several markers on their cell surfaces including CD123, CD4, and CD56. Because of its rarity and challenging clinical presentation, there was no standard of care in managing BPDCN for decades and its prognosis overall was poor. However, as understanding of this rare neoplasm has increased, so have treatment options. The conventional cytotoxic chemotherapy regimens once used in the treatment of BPDCN were modest in their impact on disease relapse until paired with hematopoietic stem cell transplant. Although recent data suggest that there still remains a role for chemotherapeutic agents, targeted modalities have expanded the overall BPDCN treatment landscape. The CD123\u2010targeted agent, tagraxofusp, was the first Food and Drug Administration\u2013approved monotherapy in the treatment of BPDCN. Since its inception, several CD123\u2010targeted and other cell\u2010surface agents have been investigated, with many agents still in the preclinical stages. Although relapsed\/refractory disease and central nervous system disease both remain formidable areas of research, there are several promising therapeutic approaches that could have a significant impact on the trajectory of treatment. This review will provide detailed insight on the novel drugs currently in use and those being explored in the management of BPDCN.<\/jats:p>","DOI":"10.1002\/cncr.34345","type":"journal-article","created":{"date-parts":[[2022,6,21]],"date-time":"2022-06-21T04:01:31Z","timestamp":1655784091000},"page":"3019-3026","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Blastic plasmacytoid dendritic cell neoplasm (<scp>BPDCN<\/scp>): A promising future in the era of targeted therapeutics"],"prefix":"10.1002","volume":"128","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3246-2897","authenticated-orcid":false,"given":"Ijele J.","family":"Adimora","sequence":"first","affiliation":[{"name":"Department of Internal Medicine The University of Texas McGovern Medical School  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathaniel 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