~60% to 90% of patients present with leukemic disease4,5
Important to know
- Primary sites of involvement include peripheral blood and bone marrow4,6
- Secondary sites of involvement include lymph nodes and viscera1,4
- In approximately 10% of cases, leukemic involvement may occur in the absence of skin disease7
- Lymphadenopathy and cytopenia due to bone marrow involvement may be present at diagnosis or may occur upon progression1
- 20% of patients present with regional lymph node involvement6
- Neurological involvement of BPDCN is present in ~10% of patients at diagnosis and appears to be more frequent after relapse (~30%)8
- Cytology, in association with flow cytometry, immunophenotyping, and clinical history, can help obtain an accurate diagnosis of BPDCN9
BPDCN may be mistaken for other hematologic malignancies, such as AML, leukemia cutis, myeloid sarcoma, NK/T-cell lymphoma, ALL, MDS, CMML, and CTCL1-4
Historical overall survival10
- Historically, median overall survival for BPDCN is approximately 8 to 14 months after diagnosis10,11
- BPDCN may rapidly progress to an aggressive leukemia4,10
AML = acute myeloid leukemia; NK = natural killer; ALL = acute lymphoblastic leukemia; MDS = myelodysplastic syndrome; CMML = chronic myelomonocytic leukemia; CTCL = cutaneous T-cell lymphoma.
- Pagano L, et al. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. Br J Haematol. 2016;174(2):188-202.
- 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.
- 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.
- Riaz W, et al. Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer Control. 2014;21(4):279-289.
- Herling M, Jones D. CD4+/CD56+ hematodermic tumor: the features of an evolving entity and its relationship to dendritic cells. Am J Clin Pathol. 2007;127(5):687-700.
- Reichard KK. Blastic plasmacytoid dendritic cell neoplasm: how do you distinguish it from acute myeloid leukemia? Surg Pathol Clin. 2013;6(4):743-765.
- Hirner JP, et al. Blastic plasmacytoid dendritic cell neoplasm: the dermatologist’s perspective. Hematol Oncol Clin North Am. 2020;34(3):501-509.
- Martín-Martín L, 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.
- Ferreira J, et al. Cytomorphological features of blastic plasmacytoid dendritic cell neoplasm on FNA and cerebrospinal fluid cytology: a review of 6 cases. Cancer Cytopathol. 2016;124(3):196-202.
- 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.