Pivekimab sunirine and the evolving treatment of blastic plasmacytoid dendritic cell neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy characterized by leukemic blasts with frequent cutaneous involvement and high CD123 expression. Pivekimab sunirine, an anti-CD123 antibody-drug conjugate with a DNA-alkylating payload, was approved by the United States Food and Drug Administration on May 27, 2026, for adults with BPDCN based on the CADENZA study. We review its mechanism, clinical efficacy, toxicity, and role in the evolving treatment landscape for BPDCN and AML.