Terapevticheskii arkhivTerapevticheskii arkhiv0040-36602309-5342LLC Obyedinennaya Redaktsiya10974410.26442/00403660.2022.07.201734Research ArticlePathomorphological and clinical characteristics of blastic plasmocytoid dendritic cell neoplasm. Case reportSherstnevAndrei A.sherstnevandrejj@mail.ruhttps://orcid.org/0000-0002-1597-4591KovriginaAlla M.sherstnevandrejj@mail.ruhttps://orcid.org/0000-0002-1082-8659SolovyovaTatiana I.sherstnevandrejj@mail.ruhttps://orcid.org/0000-0001-8246-6581MoiseevaTatiana N.sherstnevandrejj@mail.ruhttps://orcid.org/0000-0001-9591-8508National Research Center for Hematology120820229478918961108202211082022Copyright © 2022, Consilium Medicum2022<p>The paper reveals the 5 cases date about blastic plasmacytoid dendritic cell neoplasm. The presented information demonstrates morphological, immunohistochemical data and clinical manifestations.</p>blastic plasmocytoid dendritic cell neoplasmdiagnosticshistopathologyimmunohistochemistryskinбластная опухоль из плазмоцитоидных дендритных клетокдиагностикаморфологияиммуногистохимиякожа[Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues (Revised 4th edition). IARC: Lyon, 2017; p. 173-8.][Bueno C, Almeida J, Lucio P, et al. Incidence and characteristics of CD4(+)/HLA DRhi dendritic cell malignancies. Haematologica. 2004;89(1):58-69.][Wang H, Cao J, Hong X. Blastic plasmacytoid dendritic cell neoplasm without cutaneous lesion at presentation: case report and literature review. Acta Haematologica. 2012;127(2):124-7. DOI:10.1159/000334703][Trottier AM, Cerquozzi S, Owen CJ. Blastic plasmacytoid dendritic cell neoplasm: challenges and future prospects. Blood Lymphat Cancer. 2017;7:85-93. DOI:10.2147/BLCTT.S132060][Adachi M, Maeda K, Takekawa M, et al. High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma. Am J Hematol. 1994;47(4):278-82. DOI:10.1002/ajh.2830470406][Kazakov DV, Mentze T, Burg G, et al. Blastic natural killer-cell lymphoma of the skin associated with myelodysplastic syndrome or myelogenous leukaemia: a coincidence or more? Br J Dermatol. 2003;149:869-76. DOI:10.1046/j.1365-2133.2003.05639.x][Dzionek A, Inagaki Y, Okawa K, et al. Plasmacytoid dendritic cells: from specific surface markers to specific cellular functions. Hum Immunol. 2002;63(12):1133-48. DOI:10.1016/s0198-8859(02)00752-8][Saadeh D, Kurban M, Abbas O. Plasmacytoid dendritic cell role in cutaneous malignancies. J Dermatol Sci. 2016;83(1):3-9. DOI:10.1016/j.jdermsci.2016.05.008][Jegalian AG, Buxbaum NP, Facchetti F, et al. Blastic plasmacytoid dendritic cell neoplasm in children: diagnostic features and clinical implications. Haematologica. 2010;95(11):1873-9. DOI:10.3324/haematol.2010.026179][Nomburg J, Bullman S, Chung SS, et al. Comprehensive metagenomic analysis of blastic plasmacytoid dendritic cell neoplasm. Blood Adv. 2020;4(6):1006-11. DOI:10.1182/bloodadvances.2019001260][Testa U, Pelosi E, Frankel A. CD 123 is a membrane biomarker and a therapeutic target in hematologic malignancies. Biomark Res. 2014;2(1):4. DOI:10.1186/2050-7771-2-4][Bôle-Richard E, Fredon M, Biichlé S, et al. CD28/4-1BB CD123 CAR T cells in blastic plasmacytoid dendritic cell neoplasm. Leukemia. 2020;34(12):3228-41. DOI:10.1038/s41375-020-0777-1][Torres ANB, Cats D, Mei H, et al. Whole-genome analysis uncovers recurrent IKZF1 inactivation and aberrant cell adhesion in blastic plasmacytoid dendritic cell neoplasm. Genes Chromosomes Cancer. 2020;59(5):295-308. DOI:10.1002/gcc.22831][Ceribelli M, Hou ZE, Kelly PN, et al. A druggable TCF4- and BRD4-dependent transcriptional network sustains malignancy in blastic plasmacytoid dendritic cell neoplasm. Cancer Cell. 2016;30(5):764-78. DOI:10.1016/j.ccell.2016.10.002][Sukswai N, Aung PP, Yin CC, et al. Dual expression of TCF4 and CD123 is highly sensitive and specific for blastic plasmacytoid dendritic cell neoplasm. Am J Surg Pathol. 2019;43(10):1429-37. DOI:10.1097/PAS.0000000000001316][Agha M, Monaghan S, Swerdlow S. Venetoclax in a Patient with a Blastic Plasmacytoid Dendritic-Cell Neoplasm. New England J Med. 2018;379(15):1479-81. DOI:10.1056/NEJMc1808354][Aung P, Sukswai N, Nejati R, et al. PD1/PD-L1 Expression in Blastic Plasmacytoid Dendritic Cell Neoplasm. Cancers. 2019;11:695. DOI:10.3390/cancers11050695]