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.
Keywords: BPDCN; allogeneic stem cell transplantation; chemotherapies; conventional therapeutics; targeted therapies.