ICD-11 classes
03 Diseases of the blood or blood-forming organs
Neoplasms of haematopoietic or lymphoid tissues
2A60 — Acute myeloid leukaemias and related precursor neoplasms
2A60.5 — Blastic plasmacytoid dendritic cell neoplasm

ICD-11 2A60.5 — Blastic plasmacytoid dendritic cell neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive tumour derived from the precursors of plasmacytoid dendritic cells (PDCs, also called professional type I interferon–producing cells or plasmacytoid monocytes), with a high frequency of cutaneous and bone marrow involvement and leukaemic dissemination.There are currently no clues to the etiology of BPDCN, but its association with myelodysplastic syndrome (MDS) in some cases may suggest a related pathogenesis. Gene expression profiling studies have revealed that the neoplastic cells show a gene expression signature similar to that of resting normal PDCs and closer to that of myeloid than of lymphoid precursors.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Acute myeloid leukaemias and related precursor neoplasms #1596
• Miscellaneous haematological neoplasms which may arise in the skin #17479