Abstract: Objective To summarise the clinical characteristics, pathology,treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN) in children.Methods We retrospectively analysed the olinical data of 5 children with BPDCN admitted to Peking University People's Hospital from 2014 to 2018 and analyzed the follow-up statistics.The median follow-up time was 37months.Results The sex ratio is 2∶3 and 5 children manifested with cutaneous involvement at presentation. All patients confined organs including skin,others including spleen and/or liver,lymph nodes,vertebrae, peripheral blood and no central nervous system was confined. All patients confirmed by the skin or bone marrow pathology and they all expressed CD4, CD56, CD123. All children had no specific gene expression, 1 case had chromosomal abnormalities. Induction chemotherapy was performed with ALL or NHL regimen and AML regimen was not selected. All patients got remission after induction chemotherapy.All of them were followed by Allo-HSCT with 4 patients having long-term survival and 1 in treatment- related death. All patients have no disease-related death.Conclusion Children with BPDCN are less invasive than adults, and sensitive to chemotherapy.The prognosis is better for Allo-HSCT.

Key words: blastic plasmacytoid dendritic cell neoplasm, Children, clinical characteristics, Treatment