Have you seen a case of BPDCN?
- BPDCN is derived from the precursors of plasmacytoid dendritic cells (pDCs), and is characterized by an aggressive clinical course and high frequency of cutaneous and bone marrow involvement1
- Previous names for BPDCN included:
- 2001: Blastic natural killer (NK) cell lymphoma2
- 2005: CD4+/CD56+ hematodermic neoplasm2,3
- The World Health Organization (WHO) classification for BPDCN has been evolving
- 2008: BPDCN classified as a subset of acute myeloid leukemia (AML)4
- 2016: BPDCN officially classified as a unique myeloid neoplasm5
- The exact incidence of BPDCN is unknown because the nomenclature used to describe BPDCN has evolved over the years, along with the understanding of its underlying biology1
Frequent reclassification and renaming have likely contributed to the underrecognition and underdiagnosis of BPDCN1
Who are patients with BPDCN?
~75% are men6
Typically between
60 to 70 years of age,
but all ages can be affected1
Affects all races
and geographic locations1
- Primary sites of involvement include skin, bone marrow, and peripheral blood7,8
- Secondary sites of involvement include lymph nodes and viscera1,7
- A relatively high rate of central nervous system involvement is observed at diagnosis (~10%) or during relapse of disease (~30%) in patients with BPDCN presenting with neurological symptoms9
BPDCN can be hidden in plain sight
| BPDCN may be mistaken for other malignancies1,7,10,11 | |
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Similarity to other hematologic malignancies, including those with skin manifestations, may contribute to misdiagnosis1,7,10,11
| BPDCN may be mistaken for other malignancies1,7,10,11 | |
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Similarity to other hematologic malignancies, including those with skin manifestations, may contribute to misdiagnosis1,7,10,11
Skin lesions are often the first sign of BPDCN1
Nodular lesions
Nodules are often purple/violet in color and localized to various body areas, particularly the trunk, limbs, and head.7,12
Reprinted by permission from Springer Nature: Modern Pathology, Neoplasms derived from plasmacytoid dendritic cells, Facchetti F, © 2016.
Diffuse, bruise-like macules
One or several bruise-like patches confined to various body areas.7,12
Reprinted by permission of SAGE Publications, Inc.
Hyperpigmented macules
These macules, which may vary in size and distribution, may be confused with neoplastic and nonneoplastic etiologies.13
Republished with permission from American Society of Hematology.
Understanding cutaneous presentation
- ~85% to 90% of patients present with skin lesions10,12,13
- A dermatologist may be the first to identify the signs of BPDCN1
- The heterogeneous dermatologic presentation of BPDCN may result in underdiagnosis12
- Skin lesions are often nonpruritic in nature and vary in size, shape, color, and distribution7,13
- CD123 can be both a diagnostic marker and a therapeutic target in BPDCN10,14,15
- CD123, CD4, and CD56 comprise a signature marker triad that is key in identifying BPDCN1,16
- BPDCN can include other markers, such as TCL1, TCF4, and CD303 (BDCA2)1,17
- BPDCN can be diagnosed through any biopsy of malignant cells14
Consider BPDCN in your patients with hematologic cancer
BPDCN = blastic plasmacytoid dendritic cell neoplasm.
References: 1. Pagano L, et al. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. Br J Haematol. 2016;174(2):188-202. 2. Chan JK, et al. Blastic NK-cell lymphoma. In: Jaffe ES, et al., eds. WHO Classification. Tumors of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001:214-215. 3. Willemze R, et al, WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768-3785. 4. Facchetti F, et al. Blastic plasmacytoid dendritic cell neoplasm. In: Swerdlow SH, et al, eds. WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008:145-147. 5. 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. 6. Pagano L, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 2013;98(2):239-246. 7. Riaz W, et al. Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control. 2014;21(4):279-289. 8. Reichard KK. Blastic plasmacytoid dendritic cell neoplasm: how do you distinguish it from acute myeloid leukemia? Surg Pathol Clin. 2013;6(4):743-747. 9. Martín-Martín L, Almeida J, Pomares H, et al. Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central nervous system involvement at diagnosis and benefits from intrathecal therapy. Oncotarget. 2016;7(9):10174-10181. 10. 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. 11. 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. 12. Julia F, et al. Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol. 2013;169(3):579-586. 13. Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program. 2016(1):16-23. 14. Facchetti F, et al. Neoplasms derived from plasmacytoid dendritic cells. Mod Pathol. 2016;29(2):98-111. 15. 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. 16. 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 February 5, 2020. 17. Ceribelli M, Hou ZE, Kelly PN, 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.