[Clinical Analysis of Patients with Blastic Plasmacytoid Dendritic Cell Neoplasm].

To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm(BPDCN).Clinical data of 5 patients diagnosed with BPDCN in Wuhan First Hospital and Wuhan Tongji Hospital from June 2016 to November 2021 were retrospectively analyzed.Among the 5 patients, 3 were male and 2 were female, with a median age of 28(10-52) years old. Four patients showed obvious skin damage at the initial diagnosis; the other one showed clinical manifestations of acute leukemia rather than obvious skin damage at the initial diagnosis, but infiltrated skin when the disease relapsed after treatment. Other infiltration sites of lesions included bone marrow (2/5), peripheral blood (2/5), lymph nodes (3/5), liver and spleen (2/5). All patients had no clinical manifestation of central nervous system infiltration. Tumor cell specific immune markers CD4, CD56, CD123 were all positive, and the median Ki-67 index was 70%. TET2, ASXL1 and NRAS gene mutations were found respectively in 3 patients by next-generation sequencing technique (NGS). ALL-like, AML-like and invasive NK/T cell lymphoma-like first-line induction chemotherapy regimens were used for the patients. One patient died of severe complications during the early stage of chemotherapy, 3 patients were evaluated as CR, and 1 patient was evaluated as PR. 2 patients were recurred and progressed after induction of chemotherapy, and one of them was evaluated as CR after re-treatment. One patient received autologous hematopoietic stem cell transplantation (auto-HSCT) and got long-term survival (OS 87 months). 3 patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT), of which one died of transplantation related complications, and 2 cases survived. The median follow-up time of 4 patients with evaluable efficacy was 28.5(9-84) months, the median OS time was 31.5(10-87) months.BPDCN is a highly heterogeneous malignant tumor with a poor prognosis. HSCT, especially allo-HSCT can significantly improve the prognosis of BPDCN patients.母细胞性浆细胞样树突细胞肿瘤患者的临床分析.探讨母细胞性浆细胞样树突细胞肿瘤（BPDCN）的临床特征、治疗方案及预后.回顾性分析2016年6月至2021年11月在武汉市第一医院和武汉市同济医院诊治的5例BPDCN患者的临床资料.5例患者中，男性3例，女性2例，中位年龄28（10-52）岁。4例起病时有明显皮肤损害，1例以急性白血病起病，无明显皮肤损害，但在复发时累及皮肤。其他病变累及部位包括骨髓（2/5）、外周血(2/5)、淋巴结（3/5）、肝脾（2/5），无中枢神经系统受累。肿瘤细胞特征性的免疫标记物CD4、CD56、CD123均为阳性，中位Ki-67指数为70%。利用高通量测序技术（NGS）发现3例患者分别存在TET2、ASXL1、NRAS基因突变。采用急性淋巴细胞白血病（ALL）、急性髓系白血病（AML）及侵袭性NK/T细胞淋巴瘤的一线诱导化疗方案，1例早期死亡，3例达CR，1例达PR。2例患者复发、进展，更换治疗方案后其中1例再次达CR。1例行自体造血干细胞移植，长期存活，OS时间87个月；3例行异基因造血干细胞移植，1例死亡，2例存活。可评价疗效的4例患者中位随访时间为28.5（9-84）个月，中位OS时间为31.5（10-87）个月.BPDCN异质性强，总体预后不良。造血干细胞移植特别是异基因造血干细胞移植可显著改善BPDCN患者的预后.