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

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) often includes a dermatologic presentation that can be mistaken for other skin disorders. 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:


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

Republished with permission from American Society of Hematology Education Program.


One or several rash or bruise-like patches found on various body areas1,4

Overlapping symptoms and marker expression drive diagnosis in BPDCN

BPDCN is often mistaken for other malignancies with skin manifestations. Unfortunately, delay in correct diagnosis can mean that by the time BPDCN is recognized, disease progression may have already worsened the patient’s condition.3,4,6-8

BPDCN is often misdiagnosed as3,4,6,9

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

Key pathologic factors 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 your initial diagnostic panels

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

While CD4 and CD56 are often standard markers for many 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 worsened the patient’s condition.6,8

Research continues to evolve

Stemline Therapeutics, Inc. is committed to leading research advancements in BPDCN.8,12

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. 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.