Overlapping symptoms and marker expression drive diagnosis in BPDCN

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is often misdiagnosed, mistaken for AML, NHL, ALL, MDS, and CMML, as well as other malignancies with skin manifestations. Unfortunately, delay in correct diagnosis can mean that by the time BPDCN is recognized, suboptimal treatment and disease progression may have already worsened the patient’s condition.1-5

BPDCN is often misdiagnosed as1-3,6

  • AML. Because of shared markers, BPDCN may be misdiagnosed as AML
  • NHL. Both BPDCN and NHL present similarly
  • ALL. Because of shared markers, BPDCN may be misdiagnosed as ALL
  • MDS. BPDCN may be misdiagnosed as this malignancy
  • CMML. BPDCN may be misdiagnosed as this malignancy
  • Leukemia cutis, cutaneous lymphoma, NK cell neoplasms. Because patients with BPDCN often present with skin lesions, and because morphological aspects as well as histopathological features of both diseases can be diagnostically indifferent, BPDCN may be mistaken for these malignancies

Key pathologic factors of BPDCN7

BPDCN pathology morphology cytology

Prior diagnoses should be considered

Approximately 10% to 20% of patients with BPDCN have a previous history of, or may have been misdiagnosed with, the following hematologic malignancies: MDS, CMML, and AML.2,8,9

Consider adding CD123 to your current hematologic diagnostic panel

There is a clear pattern of diagnostic markers that differentiate BPDCN—the signature triad CD123, CD4, CD56.2

While CD4 and CD56 are standard parts of many diagnostic panels, CD123 is rarely included in initial diagnoses. This can lead to misdiagnosis, suboptimal treatment, and poor outcomes.2,5

BPDCN morphology cell stain CD123

There are no approved treatments or an effective standard of care for BPDCN

Standard treatments for more common hematologic cancers often prove ineffective in patients with BPDCN. Although they may produce acute responses, rapid relapse and short survival times are the most common outcomes. No treatments have been approved for BPDCN in any part of the world.2,3,5

Hematopoietic stem cell transplantation (HSCT)

Currently, HSCT appears to be the most effective treatment for patients who have achieved complete response with induction therapy, inducing durable, long-term disease control.7

Consider BPDCN in all of your patients with hematologic cancers.

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


  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. Pemmaraju N. Novel pathways and potential therapeutic strategies for blastic plasmacytoid dendritic cell neoplasm (BPDCN): CD123 and beyond [published online ahead of print October 24, 2017]. Curr Hematol Malig Rep. doi:10.1007/s11899-017-0425-7.
  7. Reichard KK. Blastic plasmacytoid dendritic cell neoplasm: how do you distinguish it from acute myeloid leukemia? Surg Pathol Clin. 2013;6(4):743-765.
  8. 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.
  9. 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.