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blastic plasmacytoid dendritic cell neoplasm (BPDCN)
Epidemiology:
- rare
- more common in men than women & in patients >= 60 years
Pathology:
- bone marrow disease & blood disease
- can affect multiple organs, including lymph nodes & skin
- often presents as leukemia or evolves into acute leukemia
- skin biopsy (case report) [2]
- diffuse dermal infiltrate with blast-like cells extending to the hypodermis
- immunohistochemistry: cells positive for CD4, CD56, & CD123
Clinical manifestations:
- may present without evidence of extracutaneous manifestations
- case report in 26 yer or man [2]
- progressive painless, violaceousl large, coalescing nonpruritic skin lesions on face, back, trunk & limbs
- no systemic symptoms, (night sweats or weight loss)
Special laboratory:
- bone marrow aspiration:
- 1% plasmacytoid dendritic cells (case report) [1]
Radiology:
- complete staging indicated
- computed tomography (whole body) for extracutaneous disease
Management:
- tagraxofusp (Elzonris) [1]
- hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high-dose methotrexate & cytarabine for 8 cycles results in complete remission (case report) [2]
- prognosis (case report) [2]
- relapse after 6 months of remission without response to further therapy
- death 27 months after diagnosis (case report) [2]
Related
dendritic cell
dendritic cell precursor type 2 (plasmacytoid dendritic cell, natural interferon (IFN)-producing cells, pre-DC2, pDC)
General
dendritic cell neoplasm
hematologic malignancy (hematopoietic malignancy, hematologic cancer)
skin cancer
bone marrow disease
References
- FDA News Release. Dec 21, 2018
FDA approves first treatment for rare blood disease.
https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm629020.htm
- Gonzaga Y, Fontes M,
Images in Clinical Medicine: Blastic Plasmacytoid Dendritic-Cell Neoplasm.
N Engl J Med 2020; 383:2158 Nov 26
PMID: 33252872
https://www.nejm.org/doi/full/10.1056/NEJMicm2003702