母细胞性浆细胞样树突状细胞肿瘤一例并文献复习

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

目的 提高对母细胞性浆细胞样树突状细胞肿瘤（BPDCN）的认识.方法 通过1例病例报告和文献复习,分析BPDCN患者的临床特点、治疗选择及预后.结果 BPDCN患者以中老年男性居多,绝大多数发病时表现为皮疹和骨髓浸润,特征性免疫表型为CD4＋、CD56＋、CD123＋.在诱导治疗方面,淋系方案比髓系方案治疗的总反应率高,复发率较低,总生存较长.异基因造血干细胞移植可能带来长期生存.患者发病时白细胞和血小板总数可能是影响BPDCN预后的危险因素.结论 BPDCN在临床表现和免疫表型上都具有显著特点,治疗方案建议以淋系方案为主,在首次完全缓解期行异基因造血干细胞移植是改善预后、获得远期生存的重要手段.

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 longterm survival.