BPDCN is an aggressive and deadly hematologic cancer

BPDCN definition

Plasmacytoid dendritic cells (pDCs) are normally produced in the bone marrow, circulate in the blood, and accumulate in lymph nodes when an immune response is triggered. BPDCN is characterized by the malignant proliferation of pDC-like cells.1

Previous names for BPDCN include acute agranular NK cell leukemia and blastic NK cell tumor. In 2008, the World Health Organization (WHO) officially named this malignancy BPDCN and classified it as a separate myeloid neoplasm.2

BPDCN has had several different names as a deeper appreciation of the underlying biology of the disease developed. Frequent reclassification and renaming has likely contributed to the underrecognition and underdiagnosis of BPDCN.3

BPDCN can occur at any age; however, most patients are older, with a median age range of 60-70 years. Approximately 75% of cases occur in men.4,5

Hidden in plain sight: BPDCN may be confused with more common hematologic cancers like AML, NHL, ALL, MDS, and CMML, as well as other malignancies with skin manifestations.1,5-7

BPDCN shares key symptoms with other types of hematologic cancers

BPDCN symptoms lesions nodules

In BPDCN, primary sites of involvement include the bone marrow, peripheral blood, and skin. Secondary sites may include lymph nodes and viscera. Comorbidities most commonly found with bone marrow involvement are neutropenia (34%), anemia (65%), and thrombocytopenia (78%).5,6

BPDCN often presents with cutaneous lesions in the form of skin nodules (73%) and bruise-like lesions (12%). These can vary in size, color, and shape, from a few millimeters to up to 10 cm.5

Lesions can be small, or pronounced and extensive, sometimes covering large areas of the body. Some patients show no signs of cutaneous lesions or rash, but instead only feature leukemic disease.6

Outcomes for BPDCN are poor

Currently, there are no agents approved by the US Food and Drug Administration (FDA) for the treatment of BPDCN, and no standard of care has been designated. Overall survival for BPDCN is approximately 8 to 14 months after diagnosis.4,5,8

Identifying and diagnosing BPDCN

Hematologist/oncologists, hematopathologists, dermatologists, and dermatopathologists all have the ability to recognize the significance of the CD123, CD4, CD56 marker triad and to diagnose BPDCN.6,9

Recognition is the first step.Stemline Therapeutics, Inc. is committed to leading research advancements in BPDCN.10,11

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


  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. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391-2405.
  3. Pemmaraju N. Blastic plasmacytoid dendritic cell neoplasm. Clin Adv Hematol Oncol. 2016;14(4):220-222.
  4. Pagano L, Valentini CG, Pulsoni A, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013;98(2):239-246.
  5. 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.
  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. 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.
  9. Reichard KK. Blastic plasmacytoid dendritic cell neoplasm: how do you distinguish it from acute myeloid leukemia? Surg Pathol Clin. 2013;6(4):743-765.
  10. 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.
  11. 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.