A Systematic Literature Review Of 233 Blastic Plasmacytoid Dendritic Cell Neoplasm Cases

Background:Blastic plasmacytoid dendritic cell neoplasms(BPDCNs)is a rare invasive hematological tumor characterized by frequent skin involvement,which prefers occurs among older male patients with poor prognosis.Due to the low incidence of this disease,there is no prospective data to support treatment options and prognostic models currently.Objects:The purpose of this article is to analyze the clinical manifestations,response to treatment regimens,survival outcomes,and disease prognosis of BPDCN patients,and summarize the current clinical treatment methods,helping guiding the trentment option of BPDCN patients with different ages more appropriately,and improving the survival and our understanding of patients.Methods:18 articles were screened from Pubmed,including 233 cases of BPDCN,in which we defined 41 patients less than or equal to 18 years of age as children patients and 192 were adult patients over 18 years of age.We compared the clinical manifestations,response to treatment regimens,survival outcomes,and disease prognosis,between adults cases and children patients of BPDCN,by analyzing published BPDCN cases.Results:(1)The distribution of affected organs in BPDCN patients at diagnosis is different among children and adults groups: adults patients are more common showing skin lesions than children patients(87.50% vs 70.73%,P=0.007).The proportion of patients who were only involved hematologic organs without skin manifestations was higher in children than that in adults patients(26.38% vs 11.46%,P=0.010).The involvement of hematologic organs associated a worse prognosis(P=0.032).(2)The complete remission rate treated with acute lymphocytic leukemia-like chemotherapy regimens had been shown to be superior to other chemotherapyregimens,such as acute myeloid leukemia-like(85.92% vs 58.06%,P=0.002),and lymphoma-like(85.92% vs 63.93%,P=0.003).(3)Age acted as an independent prognosis factor,the initial response to treatment among children patients and median overall survival were significantly better than adults patients,(89.19% vs 59.76%,P < 0.001)and(24 [2-156] months vs 12 [1-82 ] months,P < 0.0001)respectively.The relapse rate of children patients were significantly lower than that of adults patients(18.19% vs 94.90%,P < 0.001).(4)Hematopoietic stem cell transplantation(HSCT)can increase the mean overall survival time of adults patients(ALL-Like vs HSCT regimen : 24 months vs40 months,P = 0.0111),but children patients cannot benefit from HSCT(P = 0.9629).Conclusion:(1)For children and adults patients with BPDCN,the complete remission rate of ALL-like regimens is higher than that of AML and lymphoma chemotherapy.(2)Both autologous and allogeneic hematopoietic stem cell transplantation can improve the survival prognosis of adults patients,and allogeneic hematopoietic stem cell transplantation is preferred.It is not recommended to transplant immediately after the initial remission of children patients.(3)Age acts as an independent prognostic factor for patients with BPDCN,and the prognosis of adults patients is worse than that of children patients.