Blastic plasmacytoid dendritic cell neoplasm

Definition

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.

Inclusions

• blastic NK-cell lymphoma

Also indexed as

Nearby in Acute myeloid leukaemias and related precursor neoplasms

• 2A60.0Acute myeloid leukaemia with recurrent genetic abnormalities
• 2A60.1Acute myeloid leukaemia with myelodysplasia-related changes
• 2A60.2Therapy-related myeloid neoplasms
• 2A60.3Acute myeloid leukaemia, not elsewhere classified by criteria of other types
• 2A60.4Myeloid proliferation associated with Down syndrome
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified