Early diagnosis may be critical and requires a multidisciplinary team1
- Dermatologists may be the first to recognize the signs of this hematologic malignancy because ~85% to 90% of patients present with skin lesions1-4
- Pathologists are essential in diagnosing BPDCN, as correct diagnosis relies on a compatible immunophenotype, including the signature marker triad, CD123, CD4, and CD56, among other markers1,5*
- Hematologist-oncologists are key in accurately diagnosing BPDCN, differentiating it from more common cancers with similar presentations1,4,6,7
Historically, BPDCN has had poor clinical outcomes8,9
Historical overall survival8
- Historically, median overall survival for BPDCN is approximately 8 to 14 months after diagnosis8,9
- In a retrospective analysis, the mean time between the onset of lesions and the final diagnosis of BPDCN was 6.2 months2
BPDCN is currently recognized as a unique myeloid neoplasm after years of reclassification
Evolving World Health Organization classification for BPDCN
Blastic NK-cell lymphoma10
CD4+ CD56+ hematodermic neoplasm10,11
BPDCN as a subset of AML12
BPDCN as a unique myeloid neoplasm13
NK = natural killer; AML = acute myeloid leukemia.
Frequent reclassification and renaming have likely contributed to the underrecognition of BPDCN1
Who are patients with BPDCN?
The exact incidence of BPDCN is unknown because the nomenclature used to describe BPDCN has evolved over the years along with an understanding of the underlying biology.1
60 to 70
years of age,
but all ages can be affected1
Affects all races and
Have you seen a case of BPDCN?
Similarity to other hematologic malignancies may contribute to misdiagnosis.1,4,7,13 Approximately 10% to 20% of patients with BPDCN have a previous history of, or may have been misdiagnosed with, certain other hematologic malignancies, including MDS, CMML, and AML.1
BPDCN may be mistaken for
- Acute myeloid leukemia (AML)
- Leukemia cutis
- Myeloid sarcoma
- NK/T-cell lymphoma
- Acute lymphoblastic leukemia (ALL)
- Myelodysplastic syndrome (MDS)
- Chronic myelomonocytic leukemia (CMML)
- Cutaneous T-cell lymphoma (CTCL)
To help identify BPDCN, think CD1234561,5
- CD123, CD4, and CD56 comprise a signature marker triad that is key in diagnosing BPDCN1,5*
- CD123 can be both a diagnostic marker and a therapeutic target in BPDCN4,9,15
- BPDCN can be diagnosed through any biopsy of malignant cells15
*BPDCN diagnosis can include other markers, such as TCL1, TCF4, and CD303 (BDCA2).1,16Find out about BPDCN’s variable dermatologic presentation
- Pagano L, et al. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. Br J Haematol. 2016;174(2):188-202.
- Julia F, et al. Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol. 2013;169(3):579-586.
- Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program. 2016;2016(1):16-23.
- Laribi K, et al. Blastic plasmacytoid dendritic cell neoplasm: from origin of the cell to targeted therapies. Biol Blood Marrow Transplant. 2016;22(8):1357-1367.
- Pemmaraju N, Konopleva M. Treating blastic plasmacytoid dendritic cell neoplasm. The Hematologist website. http://www.hematology.org/Thehematologist/Ask/8927.aspx. Published August 28, 2018. Accessed April 25, 2019.
- Riaz W, et al. Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control. 2014;21(4):279-289.
- Goyal A, et al. Blastic plasmacytoid dendritic cell neoplasm. In: Carter JB, et al, eds. Atlas of Cutaneous Lymphomas: Classification and Differential Diagnosis. Cham, Switzerland: Springer International; 2015:193-203.
- Pagano L, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013;98(2):239-246.
- 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.
- Chan JK, et al. Blastic NK-cell lymphoma. In: Jaffe ES, et al, eds. WHO Classification. Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001:214-215.
- Willemze R, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768-3785.
- Facchetti F, et al. Blastic plasmacytoid dendritic cell neoplasm. In: Swerdlow SH, et al, eds. WHO Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008:145-147.
- Arber DA, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016; 127(20):2391-2405.
- Togami K, et al. Sex-biased ZRSR2 mutations in myeloid malignancies impair plasmacytoid dendritic cell activation and apoptosis. doi:10.1101/2020.10.29.36503.
- Facchetti F, et al. Neoplasms derived from plasmacytoid dendritic cells. Mod Pathol. 2016;29(2):98-111.
- Ceribelli M, et al. A druggable TCF4- and BRD4-dependent transcriptional network sustains malignancy in blastic plasmacytoid dendritic cell neoplasm. Cancer Cell. 2016;30(5):764-778.