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Blastic plasmacytoid dendritic cell neoplasm: challenges and future prospects

Authors Trottier AM, Cerquozzi S, Owen CJ

Received 24 August 2017

Accepted for publication 3 November 2017

Published 11 December 2017 Volume 2017:7 Pages 85—93

DOI https://doi.org/10.2147/BLCTT.S132060

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 3

Editor who approved publication: Professor David Dingli


Amy M Trottier, Sonia Cerquozzi, Carolyn J Owen

Division of Hematology and Hematological Malignancies, University of Calgary, Foothills Medical Centre, Calgary, AB, Canada

Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare CD4+ CD56+ myeloid malignancy that is challenging to diagnose and treat. BPDCN typically presents with nonspecific cutaneous lesions with or without extra-cutaneous manifestations before progressing to leukemia. Currently, there is no standard of care for the treatment of BPDCN and various approaches have been used including acute myeloid leukemia, acute lymphoblastic leukemia, and lymphoma-based regimens with or without stem cell transplantation. Despite these treatment approaches, the prognosis of BPDCN remains poor and there is a lack of prospective data upon which to base treatment decisions. Recent work examining the mutational landscape and gene expression profiles of BPDCN has identified a number of potential therapeutic targets. One such target is CD123, the α subunit of the human interleukin-3 receptor, which is the subject of intervention studies using the novel agent SL-401. Other investigational therapies include UCART123, T-cell immunotherapy, and venetoclax. Prospective trials are needed to determine the best treatment for this uncommon and aggressive neoplasm.

Keywords: BPDCN, myeloid, neoplasm, cutaneous, dendritic cell

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