~85%-90% of patients present with skin lesions1-3

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) often presents with skin lesions that may be mistaken for other skin conditions. Plasmacytoid dendritic cells (pDCs) invade the dermis where they proliferate, resulting in skin lesions of various sizes, shapes, and colors, taking the form of1,2,4,5:

Reprinted by permission from Springer Nature: Modern Pathology, Neoplasms derived from plasmacytoid dendritic cells. Facchetti F, © 2016.


Nodules localized to various body areas, particularly the trunk, limbs, and head1,4

Republished with permission from American Society of Hematology.


One or several rash or bruise-like, hyperpigmented, red-brown macules found on various body areas. Macules are often nonpruritic in nature1,2,4

Overlapping symptoms and marker expression drive diagnosis in BPDCN

BPDCN is often mistaken for other malignancies with skin manifestations. Early diagnosis is critical to the proper management of patients with BPDCN.3,4,6-8

BPDCN is often misdiagnosed as3,4,6,9

  • AML
  • Leukemia cutis
  • Cutaneous lymphoma
  • NHL
  • ALL
  • MDS
  • CMML

Key clinical and pathological features of BPDCN10

Prior diagnoses should be considered

Approximately 10% to 20% of patients with BPDCN have a previous history of, or may have been misdiagnosed with, certain hematologic malignancies including: MDS, CMML, and AML.5,6,11

Consider adding CD123 to initial diagnostic panels

There is a signature marker triad for the diagnosis of BPDCN—the key marker is CD123, in combination with CD4 and CD56.6

While CD4 and CD56 are often standard markers for many hematologic diagnostic panels, CD123 historically has not always been included in initial hematologic panels. Delay in the correct diagnosis can mean by the time BPDCN is recognized, disease progression may have already occurred.6,8,12

Research continues to evolve

Stemline Therapeutics, Inc. is committed to leading research advancements in BPDCN.

Don’t Delay Accurate Diagnosis.
Consider CD123 In initial HEMATOLOGIC Panels.6

AML = acute myeloid leukemia; NHL = non-Hodgkin’s lymphoma; ALL = acute lymphoblastic leukemia; MDS = myelodysplastic syndrome;
CMML = chronic myelomonocytic leukemia.


  1. Julia F, Petrel T, Beylot-Barry M, et al. Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol. 2013;169(3):579-586.
  2. Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program. 2016(1):16-23.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. Pemmaraju N. Novel pathways and potential therapeutic strategies for blastic plasmacytoid dendritic cell neoplasm (BPDCN): CD123 and beyond. Curr Hematol Malig Rep. 2017;12(6):510-512.
  10. Reichard KK. Blastic plasmacytoid dendritic cell neoplasm: how do you distinguish it from acute myeloid leukemia? Surg Pathol Clin. 2013;6(4):743-765.
  11. Vitte F, Fabiani B, Bénet C, et al. Specific skin lesions in chronic myelomonocytic leukemia: a spectrum of myelomonocytic and dendritic cell proliferations: a study of 42 cases. Am J Surg Pathol. 2012;36(9):1302-1316.
  12. Garnache-Ottou F, Feuillard J, Ferrand C, et al. Extended diagnostic criteria for plasmacytoid dendritic cell leukaemia. Br J Haematol. 2009;145:624–636.