Conventional Therapeutics in BPDCN Patients—Do They Still Have a Place in the Era of Targeted Therapies?

SIMPLE SUMMARY: Since its recognition in 2016 as a distinct entity among acute myeloid leukemia (AML), no consensus on treatment has been established for managing blastic dendritic cell neoplasm (BPDCN). Patients seem sensitive to standard chemotherapies, but relapses and resistance development often occur. To date, only allogeneic stem cell transplantation presents better results with extended overall survival. New targeting therapies appear regularly, offering therapeutic options. Here, we discuss the therapies currently available and the sequence of treatments that may be proposed to patients. We tried to determine the place of standard chemotherapy and allogeneic transplantation among these new targeted treatments for the BPDCN population. ABSTRACT: No benchmark treatment exists for blastic plasmacytoid dendritic cell neoplasm (BPDCN). Since the malignancy is chemo-sensitive, chemotherapy followed by hematopoietic stem cell transplantation remains an effective treatment. However, relapses frequently occur with the development of resistance. New options arising with the development of therapies targeting signaling pathways and epigenetic dysregulation have shown promising results. In this review, we focus on conventional therapies used to treat BPDCN and the novel therapeutic approaches that guide us toward the future management of BPDCN.