Blastic plasmacytoid dendritic cell neoplasm: diagnosis, manifestations, and treatment

Purpose of review Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy with historically poor outcomes. It typically manifests as asymptomatic skin lesions and cytopenias, which result from bone marrow involvement. Less commonly, it will present in lymph nodes or visceral organs as well. Although rare, BPDCN has been discussed more frequently in recent years as new drugs have been developed that could be effective at treating this disease. Recent findings Until recently, treatment for BPDCN commonly included intensive chemotherapy regimens, which are generally reserved for management of acute myeloid leukemia or acute lymphoblastic leukemia. However, in 2018 tagraxofusp (SL-401) was approved as the only treatment specifically indicated for BPDCN. Additional clinical trials are ongoing evaluating the efficacy of newer agents, which could potentially further improve the long-term outcomes for patients with BPDCN. This manuscript reviews the diagnosis, manifestations and treatment of BPDCN.