Blastic Plasmacytoid Dendritic Cell Neoplasm

**Semantic type:** Neoplastic Process

**Definition:** A clinically aggressive neoplasm derived from the precursors of plasmacytoid dendritic cells (also called professional type I interferon-producing cells or plasmacytoid monocytes), with a high frequency of cutaneous and bone marrow involvement and leukemic dissemination. (WHO 2017)

**Synonyms:** - Agranular CD4+ CD56+ Hematodermic Neoplasm/Tumor - Agranular CD4+ Natural Killer Cell Leukemia - BPDCN - Blastic NK Cell Lymphoma - Blastic NK-Cell Lymphoma - Blastic NK-cell lymphoma - Blastic Natural Killer Leukemia/Lymphoma - Blastic plasmacytoid dendritic cell neoplasm - CD4+/CD56+ Hematodermic Neoplasm - Monomorphic NK-Cell Lymphoma