Blastic plasmacytoid dendritic cell neoplasm is a rare, clinically aggressive hematologic malignancy characterized by cutaneous lesions, bone marrow involvement, and leukemic dissemination. Blastic plasmacytoid dendritic cell neoplasm terminology has evolved over decades along with the underlying pathophysiology process. It was previously known as acute agranular CD4-positive natural killer (NK) cell leukemia in 1995. After the World Health Organization (WHO) discovered that it originated from plasmacytoid dendritic cells in 2008, the term blastic plasmacytoid dendritic cell neoplasia was coined.
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