Cheng Ping, Wang Qiu-Xaing, Wang Lan-Lan, Guan Jun, Zhou Ying, Zhang Ting, Su Fei, Chen Liu-Qing, Cao Yang, Cheng Hui, Zou Liang
Department of Hematology, The First Hospital of Wuhan, Wuhan 430022, Hubei Province, China.
Department of Dermatology, The First Hospital of Wuhan, Wuhan 430022, Hubei Province, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2023 Jun;31(3):896-901. doi: 10.19746/j.cnki.issn.1009-2137.2023.03.041.
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%. , and 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.
探讨浆细胞样树突状细胞肿瘤（BPDCN）患者的临床特征、治疗及预后。
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同济医院确诊的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例患者初诊时表现为明显的皮肤损害；另1例初诊时表现为急性白血病的临床表现而非明显的皮肤损害，但治疗后疾病复发时皮肤出现浸润。病变的其他浸润部位包括骨髓（2/5）、外周血（2/5）、淋巴结（3/5）、肝脾（2/5）。所有患者均无中枢神经系统浸润的临床表现。肿瘤细胞特异性免疫标志物CD4、CD56、CD123均为阳性，Ki-67指数中位数为70%。采用二代测序技术（NGS）分别在其中3例患者中发现了 、 和 基因突变。患者采用了类似ALL、类似AML和侵袭性NK/T细胞淋巴瘤样的一线诱导化疗方案。1例患者在化疗早期死于严重并发症，3例患者评估为CR，1例患者评估为PR。2例患者诱导化疗后复发进展，其中1例再次治疗后评估为CR。1例患者接受自体造血干细胞移植（auto-HSCT）并长期生存（OS 87个月）。3例患者接受异基因造血干细胞移植（allo-HSCT），其中1例死于移植相关并发症，2例存活。4例疗效可评估患者的中位随访时间为28.5（9 - 84）个月，中位OS时间为31.5（10 - 87）个月。
BPDCN是一种高度异质性的恶性肿瘤，预后较差。造血干细胞移植，尤其是异基因造血干细胞移植可显著改善BPDCN患者的预后。
