{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T13:42:57Z","timestamp":1780926177122,"version":"3.54.1"},"reference-count":14,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2021,9,16]],"date-time":"2021-09-16T00:00:00Z","timestamp":1631750400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,9,16]],"date-time":"2021-09-16T00:00:00Z","timestamp":1631750400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Universit\u00e4tsklinikum Bonn"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Cancer Res Clin Oncol"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Blastic plasmacytoid dendritic-cell neoplasm (BPDCN) is an extremely rare disease that originates from dendritic cells and is associated with a poor overall survival (OS). Diagnostic and therapeutic standards are less well-established in comparison to other leukemic conditions and standards of care are lacking. Morphologic and molecular similarities to acute myeloid leukemia (AML), myelodysplastic syndrome (MDS) and chronic myelomonocytic leukemia (CMML) are hard to distinguish. We here report a BPDCN patient with a long, challenging diagnostic period. While bone marrow biopsies initially failed to prove the correct diagnosis, a cutaneous biopsy finally identified a CD45<jats:sup>+<\/jats:sup>\/CD56<jats:sup>+<\/jats:sup>\/CD4<jats:sup>+<\/jats:sup>\/CD123<jats:sup>+<\/jats:sup>\/CD33<jats:sup>+<\/jats:sup>\/MPO<jats:sup>\u2212<\/jats:sup> population suggestive of BPDCN which was confirmed by flow cytometry. Molecular analysis revealed an <jats:italic>ASXL-1, TET2<\/jats:italic> and <jats:italic>SRSF2<\/jats:italic>-mutation, cytogenetic analysis showed a normal karyotype. Treatment with the recently approved CD123-cytotoxin Tagraxofusp showed initially a very good response. This case reflects diagnostic and therapeutic difficulties in BPDCN as very rare, easily misdiagnosed neoplasia and the need for precise diagnostic care.<\/jats:p>","DOI":"10.1007\/s00432-021-03777-2","type":"journal-article","created":{"date-parts":[[2021,9,16]],"date-time":"2021-09-16T12:02:48Z","timestamp":1631793768000},"page":"743-748","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Blastic plasmacytoid dendritic-cell neoplasia: a challenging case report"],"prefix":"10.1007","volume":"148","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8773-8221","authenticated-orcid":false,"given":"Ruth-Miriam","family":"Koerber","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefanie A. E.","family":"Held","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Vonnahme","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3748-2184","authenticated-orcid":false,"given":"Georg","family":"Feldmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4744-5993","authenticated-orcid":false,"given":"Joerg","family":"Wenzel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2609-3095","authenticated-orcid":false,"given":"Ines","family":"G\u00fctgemann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Brossart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5298-9880","authenticated-orcid":false,"given":"Annkristin","family":"Heine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,9,16]]},"reference":[{"issue":"12","key":"3777_CR1","doi-asserted-by":"publisher","first-page":"1055","DOI":"10.1002\/ajh.23567","volume":"88","author":"K Alayed","year":"2013","unstructured":"Alayed K, Patel KP, Konoplev S, Singh RR, Routbort MJ, Reddy N, Pemmaraju N, Zhang L, Shaikh AA, Aladily TN, Jain N, Luthra R, Medeiros LJ, Khoury JD (2013) TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow. Am J Hematol 88(12):1055\u20131061. https:\/\/doi.org\/10.1002\/ajh.23567","journal-title":"Am J Hematol"},{"key":"3777_CR2","doi-asserted-by":"publisher","first-page":"58","DOI":"10.3324\/%25x","volume":"89","author":"C Bueno","year":"2004","unstructured":"Bueno C, Almeida J, Lucio P, Marco J, Garcia R, de Pablos JM, Parreira A, Ramos F, Ruiz-Cabello F, Suarez-Vilela D, San Miguel JF, Orfao A (2004) Incidence and characteristics of CD4(+)\/HLA DRhi dendritic cell malignancies. Haematologica 89:58\u201369. https:\/\/doi.org\/10.3324\/%25x","journal-title":"Haematologica"},{"issue":"24","key":"3777_CR3","doi-asserted-by":"publisher","first-page":"4238","DOI":"10.1182\/bloodadvances.2019000647","volume":"3","author":"F Garnache-Ottou","year":"2019","unstructured":"Garnache-Ottou F (2019) How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients? Blood 3(24):4238\u20134251. https:\/\/doi.org\/10.1182\/bloodadvances.2019000647","journal-title":"Blood"},{"issue":"5","key":"3777_CR4","doi-asserted-by":"publisher","first-page":"593","DOI":"10.1586\/ehm.10.51","volume":"3","author":"F Ishida","year":"2010","unstructured":"Ishida F, Kwong Y-L (2010) Diagnosis and management of natural killer-cell malignancies. Expert Rev Hematol 3(5):593\u2013602. https:\/\/doi.org\/10.1586\/ehm.10.51","journal-title":"Expert Rev Hematol"},{"issue":"6","key":"3777_CR5","doi-asserted-by":"publisher","first-page":"392","DOI":"10.1097\/PAP.0b013e3181bb6bc2","volume":"16","author":"AG Jegalian","year":"2009","unstructured":"Jegalian AG, Facchetti F, Jaffe ES (2009) Plasmacytoid dendritic cells: phys-iologic roles and pathologic states. Adv Anat Pathol 16(6):392\u2013404. https:\/\/doi.org\/10.1097\/PAP.0b013e3181bb6bc2","journal-title":"Adv Anat Pathol"},{"key":"3777_CR6","doi-asserted-by":"publisher","unstructured":"Laribi K, Denizon N, Besan\u00e7on A, Farhi J, Lemaire P, Sandrini J, Truong C, Ghnaya H, Baugier de Materre A (2016) Blastic plasmacytoid dendritic cell neoplasm: from origin of the cell to targeted therapies. Biology of Blood and Marrow Transplantation 1357\u20131367. https:\/\/doi.org\/10.1016\/j.bbmt.2016.03.022","DOI":"10.1016\/j.bbmt.2016.03.022"},{"key":"3777_CR7","doi-asserted-by":"publisher","unstructured":"Menezes J, ACquadro F, Wiseman M, G\u00f3mez-L\u00f3pez G, Salgado RN, Talavera-Casnas, Bruno I, Cerver JV, Montes-Morena S, Hern\u00e1ndez-Rivas J, Ayala R, Calasanz MJ, Larrayoz MJ, Brichs LF, Gonzalez-Vincet M, Piris MA, \u00c1lvarez A, Cigudosa JC. Exome sequencing reveals novel and recurrent mutations with clinical impact in blastic plasmacytoid dendritic cell neoplasm. Leukemia 28(4):823\u20139. https:\/\/doi.org\/10.1038\/leu.2013.283","DOI":"10.1038\/leu.2013.283"},{"issue":"2","key":"3777_CR8","doi-asserted-by":"publisher","first-page":"156","DOI":"10.1158\/2159-8290.CD-16-0999","volume":"7","author":"J Montero","year":"2017","unstructured":"Montero J, Stephansky J, Cai T, Griffin GK, Cabal-Hierro L, Togami HLJ, Galinsky I, Morgan EA, Aster JC, Davids MS, LeBoeuf NR, Stone RM, Konopleva M, Pemma N (2017) Blastic plasmacytoid dendritic cell neoplasm is dependent on BCL-2 and sensitive to venetoclax. Cancer Discov 7(2):156\u2013164. https:\/\/doi.org\/10.1158\/2159-8290.CD-16-0999","journal-title":"Cancer Discov"},{"key":"3777_CR9","doi-asserted-by":"publisher","unstructured":"Ohe R, Ye Aung N, Shiono Y, Utsunomiya A, Kabasawa T, Tamazawa N, Tamura Y, Kato T,Yamada A, Hasegawa S, Aizawa K, Inokura K, Ito S, Toubai T (2018) Detection of Minimal Bone Marrow involvement of Blastic Plasmacytoid Dendritic Cell Neoplastic Cells\u2014CD303 immunostaining as a diagnostic tool.J Clin Exp Hematop 58(1): 1\u20139. https:\/\/doi.org\/10.3960\/jslrt.17030","DOI":"10.3960\/jslrt.17030"},{"issue":"2","key":"3777_CR10","doi-asserted-by":"publisher","first-page":"239","DOI":"10.3324\/haematol.2012.072645","volume":"98","author":"L Pagano","year":"2013","unstructured":"Pagano L, Valentini CG, Pulsoni A, Fisogni S, Carluccio P, Mannelli F, Lunghi M, Pica G, Onida F, Cattaneo C, Piccaluga PP, Di Bona E, Todisco E, Musto P, Spadea A, D\u2019Arco A, Pileri S, Leone G, Amadori S, Facchetti F (2013) GIMEMA-ALWP. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica 98(2):239\u2013246. https:\/\/doi.org\/10.3324\/haematol.2012.072645","journal-title":"Haematologica"},{"issue":"16","key":"3777_CR11","doi-asserted-by":"publisher","first-page":"4020","DOI":"10.1182\/bloodadvances.2019000173","volume":"4","author":"N Pemmaraju","year":"2020","unstructured":"Pemmaraju N, Konopleva M (2020) Approval of tagraxofusp-erzs for blastic plasmacytoid dendritic cell neoplasm. Blood (blood Adv) 4(16):4020\u20134027. https:\/\/doi.org\/10.1182\/bloodadvances.2019000173","journal-title":"Blood (blood Adv)"},{"issue":"4","key":"3777_CR12","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1177\/107327481402100404","volume":"21","author":"W Riaz","year":"2014","unstructured":"Riaz W, Zhang L, Horna P, Sokol L (2014) Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control 21(4):279\u2013289. https:\/\/doi.org\/10.1177\/107327481402100404","journal-title":"Cancer Control"},{"key":"3777_CR13","unstructured":"Swerdlow SH, Campo E, Harris NL, et al (2017) WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. WHO Classification of Tumours, 4th edn, vol 2. IARC Press, France"},{"issue":"5","key":"3777_CR14","doi-asserted-by":"publisher","first-page":"937","DOI":"10.1182\/blood-2009-03-209262","volume":"114","author":"JW Vardiman","year":"2008","unstructured":"Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, Harris NL, Le Beau MM, Hellstr\u00f6m-Lindberg E, Tefferi A, The BCD (2008) revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood 114(5):937\u2013951. https:\/\/doi.org\/10.1182\/blood-2009-03-209262","journal-title":"Blood"}],"container-title":["Journal of Cancer Research and Clinical Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00432-021-03777-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00432-021-03777-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00432-021-03777-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,2,25]],"date-time":"2022-02-25T13:04:23Z","timestamp":1645794263000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00432-021-03777-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9,16]]},"references-count":14,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2022,3]]}},"alternative-id":["3777"],"URL":"https:\/\/doi.org\/10.1007\/s00432-021-03777-2","relation":{},"ISSN":["0171-5216","1432-1335"],"issn-type":[{"value":"0171-5216","type":"print"},{"value":"1432-1335","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,9,16]]},"assertion":[{"value":"13 June 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"23 August 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 September 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing financial interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Code availability"}},{"value":"Not applicable.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"Verbal informed consent was obtained prior to the interview of this patient. The participant has consented to the submission of the case report to the journal, unfortunately he died before making written consent.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to participate"}},{"value":"All authors agree to submit this manuscript.","order":6,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}}]}}