Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a hematologic cancer that has remained underdetected, primarily because of overlapping presentations and morphologically minute distinctions between other malignancies. Now, by including a triad of signature markers—CD123, CD4, and CD56—in all initial hematologic diagnostic panels, proper diagnosis is possible.1-3

BPDCN is an aggressive hematologic cancer and may present with features similar to AML, NHL, ALL, MDS, and CMML, as well as other malignancies with skin manifestations. Proper diagnosis of BPDCN helps avoid ineffective management and may improve outcomes for patients.2-6

CD123, as part of the signature marker triad in combination with CD4 and CD56, is a key marker in identifying BPDCN—and is a rapidly emerging target for therapeutic research in several hematologic cancers.3

Stemline Therapeutics, Inc. is committed to advancing therapeutic research to help patients with BPDCN.6,7

AML = acute myeloid leukemia; NHL = non-Hodgkin’s lymphoma; ALL = acute lymphoblastic leukemia; MDS = myelodysplastic syndrome; CMML = chronic myelomonocytic leukemia.
*BPDCN diagnosis can include other markers, such as CD4, CD56, TCL1, and CD303 (BDCA2).8
Left image republished with permission from Blood.


  1. Facchetti F, Cigognetti M, Fisogni S, Rossi G, Lonardi S, Vermi W. Neoplasms derived from plasmacytoid dendritic cells. Mod Pathol. 2016;29(2):98-111.
  2. Riaz W, Zhang L, Horna P, Sokol L. Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control. 2014;21(4):279-289.
  3. Pagano L, Valentini CG, Grammatico S, Pulsoni A. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. Br J Haematol. 2016;174(2):188-202.
  4. Laribi K, Denizon N, Besançon A, et al. Blastic plasmacytoid dendritic cell neoplasm: from origin of the cell to targeted therapies. Biol Blood Marrow Transplant. 2016;22(8):1357-1367.
  5. León-Martínez G, Meillón-García L, Morales-Polanco M, Soler-Montecinos L, Ortiz-Hidalgo C. Unusual morphologic presentations of blastic plasmacytoid dendritic cell neoplasm: report of two cases misdiagnosed as melanoma and leprosy. Int J Surg Pathol. 2014;22(1):76-82.
  6. Frankel AE, Woo JH, Ahn C, et al. Activity of SL-401, a targeted therapy directed to interleukin-3 receptor, in blastic plasmacytoid dendritic cell neoplasm patients. Blood. 2014;124(3):385-392.
  7. Pemmaraju N, Sweet KL, Lane AA, et al. Ongoing phase 2 clinical trial of SL-401 in patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN): stage 1 and stage 2 results. Poster presented at: 22nd Annual Congress of the European Hematology Association; June 23, 2017; Madrid, Spain.
  8. Reichard KK. Blastic plasmacytoid dendritic cell neoplasm: how do you distinguish it from acute myeloid leukemia? Surg Pathol Clin. 2013;6(4):743-765.