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Blastic Plasmacytoid Dendritic Cell Neoplasm: State of the Art and Prospects

1
Division of Diagnostic Haematopathology, European Institute of Oncology, IRCCS, Via Ripamonti 435, 20141 Milano, Italy
2
Unit of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, School of Medicine, Via Massarenti 1, 40138 Bologna, Italy
3
Division of Haematology, European Institute of Oncology, Via Ripamonti 435, 20141 Milano, Italy
4
Dermatology Unit, Department of Health and Science, University of Florence, School of Medicine, Viale Michelangiolo 104, 50100 Firenze, Italy
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Cancers 2019, 11(5), 595; https://doi.org/10.3390/cancers11050595
Received: 20 March 2019 / Revised: 16 April 2019 / Accepted: 25 April 2019 / Published: 28 April 2019
(This article belongs to the Special Issue Tumour Associated Dendritic Cells)
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Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare tumour, which usually affects elderly males and presents in the skin with frequent involvement of the bone-marrow, peripheral blood and lymph nodes. It has a dismal prognosis, with most patients dying within one year when treated by conventional chemotherapies. The diagnosis is challenging, since neoplastic cells can resemble lymphoblasts or small immunoblasts, and require the use of a large panel of antibodies, including those against CD4, CD56, CD123, CD303, TCL1, and TCF4. The morphologic and in part phenotypic ambiguity explains the uncertainties as to the histogenesis of the neoplasm that led to the use of various denominations. Recently, a series of molecular studies based on karyotyping, gene expression profiling, and next generation sequencing, have largely unveiled the pathobiology of the tumour and proposed the potentially beneficial use of new drugs. The latter include SL-401, anti-CD123 immunotherapies, venetoclax, BET-inhibitors, and demethylating agents. The epidemiologic, clinical, diagnostic, molecular, and therapeutic features of BPDCN are thoroughly revised in order to contribute to an up-to-date approach to this tumour that has remained an orphan disease for too long. View Full-Text
Keywords: blastic plasmacytoid dendritic cell neoplasm; clinics; morphology; phenotype; gene expression profile; mutational landscape; chemotherapy; targeted therapy blastic plasmacytoid dendritic cell neoplasm; clinics; morphology; phenotype; gene expression profile; mutational landscape; chemotherapy; targeted therapy
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
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Sapienza, M.R.; Pileri, A.; Derenzini, E.; Melle, F.; Motta, G.; Fiori, S.; Calleri, A.; Pimpinelli, N.; Tabanelli, V.; Pileri, S. Blastic Plasmacytoid Dendritic Cell Neoplasm: State of the Art and Prospects. Cancers 2019, 11, 595.

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