Blastic plasmacytoid dendritic cell neoplasm: a case report and literature review

Objective To provide new information for treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN). Methods Through one case report and literature review of 48 BPDCN cases were reviewed retrospectively. The clinical characteristics, treatment choices and prognosis were analyzed. Results BPDCN patients were mainly elderly males, mostly presented as skin rash and bone marrow infiltration. Immunophenotype was characteristically expressed as CD4, CD56 and CD123. Lymphoid-like regimens could induce higher response rate, lower relapse rate and longer overall survival compared with myeloid-like regimens. Allogeneic hematopoietic stem cell transplantation may provide long-term survival. At the onset of the disease, The counts of white blood cells(WBC)and blood platelet(Plt)may be correlated with inferior overall survival. Conclusions BPDCN is a disease with distinct clinical characteristics and immunophenotype. Lymphoid-like regimen may be the better treatment of choice, while allogeneic hematopoietic stem cell transplantation should be taken into account in the first complete remission for long-term survival. Key words: Dendritic cell neoplasm; Blastic; Plasmacytoid dendritic cells