儿童母细胞性浆细胞样树突细胞肿瘤5例病例系列报告

目的 分析儿童母细胞性浆细胞样树突细胞肿瘤(BPDCN)的临床特征、病理表现、治疗和预后。方法 纳入2014年1月1日至2019年1月31日北京大学人民医院收治的5例BPDCN患儿,截取患儿的一般资料、起病年龄、确诊年龄、临床表现、辅助检查结果、治疗情况和随访资料。结果 5例BPDCN患儿纳入本文分析,男2例,女3例,诊断年龄8~14岁。中位随访时间37(15~51)月。5例患儿起病时均有皮肤受累,皮肤外受累器官包括肝脾、淋巴结、椎体、外周血,未发现中枢神经系统受累。5例患儿均经皮肤或骨髓病理检查确诊,均表达CD4、CD56和CD123,均无特异性基因表达,1例染色体核型检测异常。诱导化疗采用ALL或NHL方案,均诱导缓解,2例NHL方案巩固化疗中出现局灶复发。5例患儿后续均行异基因造血干细胞移植,目前无病存活4例,1例为治疗相关死亡,5例患儿均无疾病相关死亡。结论 儿童BPDCN临床侵袭性弱于成人,诱导化疗敏感,后续行异基因造血干细胞移植预后较好。

Clinical analysis of 5 cases of blastic plasmacytoid dendritic cell neoplasm in children

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.