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ISSN: 2157-7013

Journal of Cell Science & Therapy
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Review Article

Blastic Plasmacytoid Dendritic Cell Neoplasm: A Review of Diagnosis, Pathology, and Therapy

Roman Shapiro1, Nikhil Sangle2, Mike Keeney3, Ian H. Chin-Yee4, Cyrus C. Hsia4and Selay Lam5,*
1Internal Medicine resident, London Health Science Centre, London ON Canada
2Department of Pathology, London Health Science Centre, London ON Canada
3Lawson Health Research Institute, London ON Canada
4Division of Hematology, Department of Medicine, London Health Science Centre, London ON Canada
5UWO Division of Hematology, London Health Sciences Centre (LHSC), Victoria Hospital 800 Commissioners Rd. East, London, Ontario N6A 5W9, USA
Corresponding Author : Dr. Selay Lam
UWO Division of Hematology
London Health Sciences Centre (LHSC)
Victoria Hospital 800 Commissioners Rd. East
London, Ontario N6A 5W9, USA
Tel: 519-685-8827
Fax: 519-685-8294
E-mail: [email protected]
Received August 31, 2015; Accepted September 25, 2015; Published September 28, 2015
Citation:Shapiro R, Sangle N, Keeney M, Chin-Yee IH, Hsia CC, et al. (2015) Blastic Plasmacytoid Dendritic Cell Neoplasm: A Review of Diagnosis, Pathology, and Therapy. J Cell Sci Ther S8:008. doi:10.4172/2157-7013.S8-008
Copyright: © 2015 Shapiro R, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignancy of dendritic cell precursors classified as a subset of acute myeloid leukemia according to WHO. It commonly presents with dermal infiltration of plasmatoid dendritic cells that have the morphologic appearance of medium-sized blasts with irregular nuclei, faint chromatin, at least one nucleolus per cell and scant cytoplasm, expressing a CD4+CD56+CD123+lin- immunophenotype. Patients typically have discoloured cutaneous lesions that grow in size, and the diagnosis of BPDCN is confirmed on skin biopsy showing the characteristic cells. Bone marrow involvement is a common feature of this neoplasm and is found in most patients at diagnosis. There is no consensus on the most appropriate treatment for BPDCN, with the neoplasm showing initial good response to high intensity chemotherapy but inevitable relapse into a more chemotherapy-resistant disease. Hematopoietic stem cell transplant in patients who achieve their first complete remission with chemotherapy is a promising therapeutic modality requiring a prospective clinical trial to evaluate its efficacy.

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