{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T17:39:05Z","timestamp":1793122745863,"version":"4.5.0"},"reference-count":73,"publisher":"Harborside Press, LLC","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with an aggressive clinical course and poor prognosis. BPDCN is most often characterized by its presentation with distinct cutaneous lesions. Bone marrow involvement, lymphadenopathy, splenomegaly, and\/or cytopenias are also seen to varying degrees. BPDCN presents with diffuse, monomorphous blasts with irregular nuclei, fine chromatin, and scant, agranular cytoplasm. Expression of CD4, CD56, and CD123 is the hallmark of BPDCN. The presence of \u22654 of CD4, CD56, CD123, TCL1, TCF4, and CD303 is necessary for the diagnosis of BPDCN. Prior to December 2018, management of BPDCN revolved around intensive chemotherapy using acute myeloid leukemia or acute lymphoblastic leukemia regimens. However, responses were transient with poor overall survival (OS). Allogeneic stem cell transplantation (alloSCT) is the only potentially curative treatment for BPDCN. Even so, only a minority of patients are candidates for alloSCT given the preponderance of disease in older individuals. For the few fit patients who are candidates for alloSCT, the aim is to achieve complete remission prior to alloSCT. Tagraxofusp (SL-401), a recombinant fusion protein containing interleukin-3 fused to truncated diphtheria toxin, was the first approved CD123-targeted therapy for BPDCN based on a phase I\/II clinical trial showing a 90% overall response rate. It was approved by the FDA on December 21, 2018. Capillary leak syndrome is an important adverse effect of tagraxofusp that requires close monitoring. Several clinical trials are underway to study other regimens for the treatment of BPDCN, including IMGN632 (pivekimab sunirine), venetoclax (alone and in combination with hypomethylating agents), CAR-T cells, and bispecific monoclonal antibodies.<\/jats:p>","DOI":"10.6004\/jnccn.2023.7026","type":"journal-article","created":{"date-parts":[[2023,5,8]],"date-time":"2023-05-08T15:50:40Z","timestamp":1683561040000},"page":"515-521","source":"Crossref","is-referenced-by-count":42,"title":["Blastic Plasmacytoid Dendritic Cell Neoplasm"],"prefix":"10.6004","volume":"21","author":[{"given":"Akriti","family":"Jain","sequence":"first","affiliation":[{"name":"University of South Florida, Morsani College of Medicine, Tampa, Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kendra","family":"Sweet","sequence":"additional","affiliation":[{"name":"Moffitt Cancer Center and Research Institute, Tampa, Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"4047","reference":[{"key":"B1","series-title":"Blood","first-page":"4591","article-title":"Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion","volume":"118","author":"Lucioni M","year":"2011","unstructured":"Lucioni M, Novara F, Fiandrino G,  Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion. Blood 2011;118:4591\u20134594."},{"key":"B2","series-title":"Haematologica","first-page":"239","article-title":"Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study","volume":"98","author":"Pagano L","year":"2013","unstructured":"Pagano L, Valentini CG, Pulsoni A,  Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica 2013;98:239\u2013246."},{"key":"B3","series-title":"Cancer","first-page":"2474","article-title":"Acute agranular CD4-positive natural killer cell leukemia","volume":"75","author":"Brody JP","year":"1995","unstructured":"Brody JP, Allen S, Schulman P,  Acute agranular CD4-positive natural killer cell leukemia. Cancer 1995;75:2474\u20132483."},{"key":"B4","series-title":"Blood","first-page":"3210","article-title":"Identification of a leukemic counterpart of the plasmacytoid dendritic cells","volume":"97","author":"Chaperot L","year":"2001","unstructured":"Chaperot L, Bendriss N, Manches O,  Identification of a leukemic counterpart of the plasmacytoid dendritic cells. Blood 2001;97:3210\u20133217."},{"key":"B5","series-title":"J Exp Med","first-page":"1101","article-title":"The enigmatic plasmacytoid T cells develop into dendritic cells with interleukin (IL)-3 and CD40-ligand","volume":"185","author":"Grouard G","year":"1997","unstructured":"Grouard G, Rissoan MC, Filgueira L,  The enigmatic plasmacytoid T cells develop into dendritic cells with interleukin (IL)-3 and CD40-ligand. J Exp Med 1997;185:1101\u20131111."},{"key":"B6","series-title":"Am J Surg Pathol","first-page":"1223","article-title":"Blastic natural killer cell leukemia\/lymphoma","volume":"21","author":"DiGiuseppe JA","year":"1997","unstructured":"DiGiuseppe JA, Louie DC, Williams JE,  Blastic natural killer cell leukemia\/lymphoma. Am J Surg Pathol 1997;21:1223\u20131230."},{"key":"B7","series-title":"Am J Surg Pathol","first-page":"852","article-title":"\u2018Agranular CD4+ CD56+ hematodermic neoplasm\u2019 (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes","volume":"26","author":"Petrella T","year":"2002","unstructured":"Petrella T, Comeau MR, Maynadi\u00e9 M,  \u2018Agranular CD4+ CD56+ hematodermic neoplasm\u2019 (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes. Am J Surg Pathol 2002;26:852\u2013862."},{"key":"B8","series-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","year":"2008","unstructured":"Swerdlow SH, Campo E, Harris NL, et al, eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th ed. IARC Publications; 2008."},{"key":"B9","series-title":"Blood","first-page":"2391","article-title":"The 2016 revision to the WHO classification of myeloid neoplasms and acute leukemia","volume":"127","author":"Arber DA","year":"2016","unstructured":"Arber DA, Orazi A, Hasserjian R,  The 2016 revision to the WHO classification of myeloid neoplasms and acute leukemia. Blood 2016;127:2391\u20132405."},{"key":"B10","series-title":"Leukemia","first-page":"1703","article-title":"The 5th edition of the WHO Classification of Haematolymphoid Tumours: Myeloid and Histiocytic\/Dendritic Neoplasms","volume":"36","author":"Khoury JD","year":"2022","unstructured":"Khoury JD, Solary E, Abla O,  The 5th edition of the WHO Classification of Haematolymphoid Tumours: Myeloid and Histiocytic\/Dendritic Neoplasms. Leukemia 2022;36:1703\u20131719."},{"key":"B11","series-title":"Leuk Res","first-page":"21","article-title":"Epidemiology and survival of blastic plasmacytoid dendritic cell neoplasm","volume":"73","author":"Guru Murthy GS","year":"2018","unstructured":"Guru Murthy GS, Pemmaraju N, Atallah E. Epidemiology and survival of blastic plasmacytoid dendritic cell neoplasm. Leuk Res 2018;73:21\u201323."},{"key":"B12","series-title":"Immunology","first-page":"3","article-title":"Human dendritic cell subsets: an update","volume":"154","author":"Collin M","year":"2018","unstructured":"Collin M, Bigley V. Human dendritic cell subsets: an update. Immunology 2018;154:3\u201320."},{"key":"B13","series-title":"Immunity","first-page":"37","article-title":"Plasmacytoid dendritic cells: development, regulation, and function","volume":"50","author":"Reizis B","year":"2019","unstructured":"Reizis B. Plasmacytoid dendritic cells: development, regulation, and function. Immunity 2019;50:37\u201350."},{"key":"B14","series-title":"Nat Med","first-page":"919","article-title":"Plasmacytoid monocytes migrate to inflamed lymph nodes and produce large amounts of type I interferon","volume":"5","author":"Cella M","year":"1999","unstructured":"Cella M, Jarrossay D, Facchetti F,  Plasmacytoid monocytes migrate to inflamed lymph nodes and produce large amounts of type I interferon. Nat Med 1999;5:919\u2013923."},{"key":"B15","series-title":"Immunol Rev","first-page":"142","article-title":"Unraveling the functions of plasmacytoid dendritic cells during viral infections, autoimmunity, and tolerance","volume":"234","author":"Swiecki M","year":"2010","unstructured":"Swiecki M, Colonna M. Unraveling the functions of plasmacytoid dendritic cells during viral infections, autoimmunity, and tolerance. 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J Clin Oncol 2007;25:3109\u20133115."},{"key":"B36","series-title":"Curr Opin Hematol","first-page":"103","article-title":"Blastic plasmacytoid dendritic cell neoplasm: diagnosis, manifestations, and treatment","volume":"27","author":"Sweet K","year":"2020","unstructured":"Sweet K. Blastic plasmacytoid dendritic cell neoplasm: diagnosis, manifestations, and treatment. Curr Opin Hematol 2020;27:103\u2013107."},{"key":"B37","series-title":"Leukemia","first-page":"2590","article-title":"Recurrent 8q24 rearrangement in blastic plasmacytoid dendritic cell neoplasm: association with immunoblastoid cytomorphology, MYC expression, and drug response","volume":"32","author":"Sakamoto K","year":"2018","unstructured":"Sakamoto K, Katayama R, Asaka R,  Recurrent 8q24 rearrangement in blastic plasmacytoid dendritic cell neoplasm: association with immunoblastoid cytomorphology, MYC expression, and drug response. 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Expert Opin Biol Ther 2020;20:115\u2013123."},{"key":"B61","series-title":"J Clin Oncol","first-page":"3032","article-title":"Long-term benefits of tagraxofusp for patients with blastic plasmacytoid dendritic cell neoplasm","volume":"40","author":"Pemmaraju N","year":"2022","unstructured":"Pemmaraju N, Sweet K, Stein AS,  Long-term benefits of tagraxofusp for patients with blastic plasmacytoid dendritic cell neoplasm. J Clin Oncol 2022;40:3032\u20133036."},{"key":"B62","series-title":"Blood","first-page":"1373","article-title":"Central nervous system involvement in blastic plasmacytoid dendritic cell neoplasm","volume":"138","author":"Pemmaraju N","year":"2021","unstructured":"Pemmaraju N, Wilson NR, Khoury JD,  Central nervous system involvement in blastic plasmacytoid dendritic cell neoplasm. Blood 2021;138:1373\u20131377."},{"key":"B63","series-title":"Blood Adv","first-page":"3027","article-title":"Characteristics and outcomes of patients with blastic plasmacytoid dendritic cell neoplasm treated with frontline HCVAD","volume":"6","author":"Pemmaraju N","year":"2022","unstructured":"Pemmaraju N, Wilson NR, Garcia-Manero G,  Characteristics and outcomes of patients with blastic plasmacytoid dendritic cell neoplasm treated with frontline HCVAD. Blood Adv 2022;6:3027\u20133035."},{"key":"B64","article-title":"First-line pivekimab sunirine elicits favorable responses in BPDCN","author":"Sava J","unstructured":"Sava J. First-line pivekimab sunirine elicits favorable responses in BPDCN. Accessed November 1, 2022. Available at: https:\/\/www.targetedonc.com\/view\/first-line-pivekimab-sunirine-elicits-favorable-responses-in-bpdcn"},{"key":"B65","series-title":"Cancer Discov","first-page":"156","article-title":"Blastic plasmacytoid dendritic cell neoplasm is dependent on BCL2 and sensitive to venetoclax","volume":"7","author":"Montero J","year":"2017","unstructured":"Montero J, Stephansky J, Cai T,  Blastic plasmacytoid dendritic cell neoplasm is dependent on BCL2 and sensitive to venetoclax. Cancer Discov 2017;7:156\u2013164."},{"key":"B66","series-title":"Am J Hematol","first-page":"401","article-title":"Clinical experience with the BCL2-inhibitor venetoclax in combination therapy for relapsed and refractory acute myeloid leukemia and related myeloid malignancies","volume":"93","author":"DiNardo CD","year":"2018","unstructured":"DiNardo CD, Rausch CR, Benton C,  Clinical experience with the BCL2-inhibitor venetoclax in combination therapy for relapsed and refractory acute myeloid leukemia and related myeloid malignancies. Am J Hematol 2018;93:401\u2013407."},{"key":"B67","series-title":"Blood","article-title":"Pre-clinical studies of anti-CD123 CAR-T cells for the treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN)","volume":"128","author":"Cai T","year":"2016","unstructured":"Cai T, Galetto R, Gouble A,  Pre-clinical studies of anti-CD123 CAR-T cells for the treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Blood 2016;128:Abstract 4039."},{"key":"B68","series-title":"Blood","article-title":"Remissions of acute myeloid leukemia and blastic plasmacytoid dendritic cell neoplasm following treatment with CD123-specific CAR T cells: a first-in-human clinical trial","volume":"130","author":"Budde L","year":"2017","unstructured":"Budde L, Song JY, Kim Y,  Remissions of acute myeloid leukemia and blastic plasmacytoid dendritic cell neoplasm following treatment with CD123-specific CAR T cells: a first-in-human clinical trial. Blood 2017;130(Suppl 1):Abstract 811."},{"key":"B69","series-title":"Clin Lymphoma Myeloma Leuk","first-page":"e579","article-title":"Combination of tagraxofusp and azacitidine is an effective option for relapsed blastic plasmacytoid dendritic cell neoplasm after allogeneic hematopoietic stem-cell transplantation","volume":"21","author":"Wang SY","year":"2021","unstructured":"Wang SY, Thomassen K, Kurch L,  Combination of tagraxofusp and azacitidine is an effective option for relapsed blastic plasmacytoid dendritic cell neoplasm after allogeneic hematopoietic stem-cell transplantation. Clin Lymphoma Myeloma Leuk 2021;21:e579\u2013582."},{"key":"B70","series-title":"Haematologica","first-page":"e432","article-title":"Daratumumab for treatment of blastic plasmacytoid dendritic cell neoplasm. A single-case report","volume":"104","author":"Iversen KF","year":"2019","unstructured":"Iversen KF, Holdgaard PC, Preiss B,  Daratumumab for treatment of blastic plasmacytoid dendritic cell neoplasm. A single-case report. Haematologica 2019;104:e432\u2013433."},{"key":"B71","series-title":"Clin Cancer Res","first-page":"6163","article-title":"Therapeutic effect of lenalidomide in a novel xenograft mouse model of human blastic NK cell lymphoma\/blastic plasmacytoid dendritic cell neoplasm","volume":"17","author":"Agliano A","year":"2011","unstructured":"Agliano A, Martin-Padura I, Marighetti P,  Therapeutic effect of lenalidomide in a novel xenograft mouse model of human blastic NK cell lymphoma\/blastic plasmacytoid dendritic cell neoplasm. Clin Cancer Res 2011;17:6163\u20136173."},{"key":"B72","series-title":"Haematologica","first-page":"1861","article-title":"Bortezomib as a new therapeutic approach for blastic plasmacytoid dendritic cell neoplasm","volume":"102","author":"Philippe L","year":"2017","unstructured":"Philippe L, Ceroi A, B\u00f4le-Richard E,  Bortezomib as a new therapeutic approach for blastic plasmacytoid dendritic cell neoplasm. Haematologica 2017;102:1861\u20131868."},{"key":"B73","series-title":"Hematol Oncol","first-page":"487","article-title":"The lenalidomide\/bortezomib\/dexamethasone regimen for the treatment of blastic plasmacytoid dendritic cell neoplasm","volume":"37","author":"Marmouset V","year":"2019","unstructured":"Marmouset V, Joris M, Merlusca L,  The lenalidomide\/bortezomib\/dexamethasone regimen for the treatment of blastic plasmacytoid dendritic cell neoplasm. 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