Blastic plasmacytoid dendritic cell neoplasm
ORPHA:86870· ICD-10 C86.4
Definition
A rare and highly aggressive myeloid malignancy characterized by predominant cutaneous involvement with concomitant or subsequent bone marrow, lymph node and visceral involvement, as well as central nervous system (CNS) disease. It derives from the precursors of plasmacytoid dendritic cells (pDC).
- Prevalence
- 1-5 / 10 000
- Inheritance
- Not applicable
- Age of onset
- Elderly