blastic plasmacytoid dendritic cell neoplasm (BPDCN)
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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)
Diagnostic procedures
- 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]
More general terms
Additional terms
- dendritic cell
- dendritic cell precursor type 2 (plasmacytoid dendritic cell, natural interferon (IFN)-producing cells, pre-DC2, pDC)
References
- ↑ Jump up to: 1.0 1.1 1.2 FDA News Release. Dec 21, 2018 FDA approves first treatment for rare blood disease. https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm629020.htm
- ↑ Jump up to: 2.0 2.1 2.2 2.3 2.4 2.5 Gonzaga Y, Fontes M, Images in Clinical Medicine: Blastic Plasmacytoid Dendritic-Cell Neoplasm. N Engl J Med 2020; 383:2158 Nov 26 PMID: https://www.ncbi.nlm.nih.gov/pubmed/33252872 https://www.nejm.org/doi/full/10.1056/NEJMicm2003702