Blastic plasmacytoid dendritic cell neoplasm

Description

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.

Index terms

Blastic plasmacytoid dendritic cell neoplasm
agranular CD4+/CD56+ haematodermic neoplasm
agranular CD4+ NK leukaemia

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blastic NK-cell leukaemia/lymphoma
blastic NK-cell lymphoma

Inclusions

blastic NK-cell lymphoma

Exclusions from above levels

2E03 – Malignant neoplasm metastasis in bone or bone marrow
http://id.who.int/icd/entity/501778925 – Malignant neoplasm metastasis in lymph nodes
http://id.who.int/icd/entity/97145147 – Malignant neoplasm metastasis in thymus gland

Sibling codes

2A60.0 – Acute myeloid leukaemia with recurrent genetic abnormalities
2A60.1 – Acute myeloid leukaemia with myelodysplasia-related changes
2A60.2 – Therapy-related myeloid neoplasms
2A60.3 – Acute myeloid leukaemia, not elsewhere classified by criteria of other types
2A60.4 – Myeloid proliferation associated with Down syndrome
2A60.Y – Other specified acute myeloid leukaemias and related precursor neoplasms
2A60.Z – Acute myeloid leukaemias and related precursor neoplasms, unspecified

Foundation entity

Blastic plasmacytoid dendritic cell neoplasm – http://id.who.int/icd/entity/783045723

Allowed postcoordination axes

Part of: 2A60: Acute myeloid leukaemias and related precursor neoplasms › 02: Neoplasms