Abstract
The purpose of this review was to summarize the clinical, diagnostic, and therapeutic features of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Several case reports and series revealed new clinical, molecular, diagnostic, and therapeutic aspects of the disease. The clinical presentation diversity has been confirmed, with frequent leukemic non-cutaneous or rare atypical manifestations. The clonal evolution in the development of BPDCN has not been sufficiently elucidated. Although certain immunophenotypic markers (CD4, TCL1, CD123, CD56, CD303) are indicative of BPDCN, the diagnosis remains in certain cases challenging. Adult (ALL)-type chemotherapy followed by hematopoietic stem cell transplantation (HSCT) is related to a favorable outcome, while chemotherapy alone seems enough in children. Future studies should continue to investigate whether CD123-directed therapies could be utilized. BPDCN is a rare aggressive malignancy that needs an aggressive induction therapy. Although a diagnostic consensus is still lacking, and large retrospective studies are also needed to obtain standardized treatment guidelines, the future perspectives are encouraging, because of novel therapeutic agents that are under investigation.
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