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Review
. 2019 Apr 28;11(5):595.
doi: 10.3390/cancers11050595.

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

Maria Rosaria Sapienza  1 , Alessandro Pileri  2 , Enrico Derenzini  3 , Federica Melle  4 , Giovanna Motta  5 , Stefano Fiori  6 , Angelica Calleri  7 , Nicola Pimpinelli  8 , Valentina Tabanelli  9 , Stefano Pileri  10
Affiliations
Review

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

Maria Rosaria Sapienza et al. Cancers (Basel). .

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.

Keywords: blastic plasmacytoid dendritic cell neoplasm; chemotherapy; clinics; gene expression profile; morphology; mutational landscape; phenotype; targeted therapy.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Examples of cutaneous manifestations of BPDCN (a) Erythematous-cyanotic plaques on the back of a patient with widespread disease. (b) Erythematous-purplish single nodule on the leg of a patient with localized disease.
Figure 2
Figure 2
Morphological findings in BPDCN. (a) Skin involvement, low-power Giemsa stain (original magnification 40×): a diffuse, monomorphous infiltrate massively involves the dermis, without epidermotropism. (b) The neoplastic cells are medium-sized blasts with fine chromatin and scanty cytoplasm, agranular on Giemsa staining (original magnification 600×).
Figure 3
Figure 3
Most common phenotypic findings in BPDCN. Tumor cells show immunoreactivity for CD4 (a), CD56 (b), CD123 (c), and CD303 (d) (original magnification 400×).
See this image and copyright information in PMC

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