Immunotherapies Targeting CD123 for Blastic Plasmacytoid Dendritic Cell Neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, difficult-to-diagnose, highly aggressive myeloid malignancy with poor prognosis and no standard of care. The interleukin-3 receptor alpha, or CD123, is highly expressed in patients with myeloid malignancies, particularly acute myeloid leukemia and BPDCN. CD123 is overexpressed on leukemic stem cells compared with normal hematopoietic stem cells, suggesting CD123 as an attractive immunotherapeutic target. To date, multiple CD123-targeted therapeutic avenues have been explored to treat BPDCN and other CD123(+) hematologic malignancies. This review summarizes immunotherapies targeting CD123 for the treatment of BPDCN and related neoplasms.