Blastic plasmacytoid dendritic cell…

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Blastic plasmacytoid dendritic cell neoplasm

ORPHA:86870Disease

Also called BPDCN

What it is

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).

Key facts

Prevalence
1-5 / 10 000 (Europe)
Age of onset
Elderly
Inheritance
Not applicable
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

ICD-10 codes

C86.4ICD-10 names this disease exactly

Cross-references

GARD 10556MONDO 0019467MONDO 19467UMLS C1301363

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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