UNDERSTANDING BPDCN

BPDCN is an aggressive hematologic cancer

BPDCN definition

 

Previous names for BPDCN included Blastic NK cell leukemia/lymphoma and Agranular CD4+/CD56+ hematodermic neoplasm. The World Health Organization (WHO) officially named this malignancy BPDCN in 2008 and subsequently classified it as a separate myeloid neoplasm in 2016. Frequent reclassification and renaming has likely contributed to the underrecognition and underdiagnosis of BPDCN.1-4

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.5

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.2,6

Hidden in plain sight: BPDCN may be confused with more common hematologic CANCERS, INCLUDING AML, NHL, ALL, MDS, and CMML, as well as other malignancies with skin manifestations.1,2,5,7

BPDCN shares key symptoms with other types of hematologic cancers

In BPDCN, primary sites of involvement include bone marrow, peripheral blood, and skin. Secondary sites may include lymph nodes and viscera. Bone marrow involvement can lead to neutropenia, anemia, and/or thrombocytopenia.1,2

BPDCN can present with cutaneous lesions in the form of nodular lesions and rash or bruise-like macules. These can vary in size, color, and shape.2,8

Lesions can be small, or pronounced and extensive, sometimes covering large areas of the body. Some patients show no signs of skin manifestations, but instead only feature leukemic disease.1

Identifying and diagnosing BPDCN

It is important for hematologist-oncologists, hematopathologists, dermatologists, and dermatopathologists to recognize the significance of the key marker CD123, in combination with CD4 and CD56 to form the signature marker triad, and to diagnose BPDCN.1,9

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.

References:

  1. 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.
  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. 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.
  4. Pemmaraju N. Blastic plasmacytoid dendritic cell neoplasm. Clin Adv Hematol Oncol. 2016;14(4):220-222.
  5. 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.
  6. 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.
  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. 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.
  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.