Zhang Xiao-Li, Liu Bing, Li Nan, Li Lu-Ke, Ji Xuan-Jing, Zhou Xue-Fang, Wang Min-Fang, Xu Hui-Li
Hematology Department, Pingdingshan First People's Hospital, Pingdingshan 467021, Henan Province, China.
Hematology Department, General Hospital of Pingmei Shenma Medical Group, Pingdingshan 467002, Henan Province, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2023 Feb;31(1):254-260. doi: 10.19746/j.cnki.issn.1009-2137.2023.01.040.
To explore the clinical manifestations, diagnosis, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN).
The clinical features, bone marrow morphology and immunophenotyping, treatment and prognosis of 4 patients with BPDCN were analyzed retrospectively.
4 patients had bone marrow， spleen and lymph nodes involvement， 2 patients had skin lesions， and 3 patients had central nervous system infiltration． Tailing phenomenon of abnormally cells could be seen in bone marrow. The immunophenotyping showed that CD56, CD4 and CD123 expression was observed in 4 patients， and CD304 in 3 patients． One patient refused chemotherapy and died early. Both patients achieved complete remission after the initial treatment with DA+VP regimen, 1 of them achieved complete remission after recurrence by using the same regimen again． One patient failed to respond to reduced dose of DA+VP chemotherapy, and then achieved complete remission with venetoclax+azacitidine.
The malignant cells in BPDCN patients often infiltrate bone marrow, spleen and lymph nodes, and have specical phenotypes, with poor prognosis. The treatment should take into account both myeloid and lymphatic systems. The treatment containing new drugs such as BCL-2 inhibitors combined with demethylation drugs is worth trying.
探讨浆细胞样树突状细胞肿瘤（BPDCN）的临床表现、诊断、治疗及预后。
回顾性分析4例BPDCN患者的临床特征、骨髓形态及免疫表型、治疗及预后情况。
4例患者均有骨髓、脾脏及淋巴结受累，2例有皮肤病变，3例有中枢神经系统浸润。骨髓中可见异常细胞的拖尾现象。免疫表型分析显示，4例患者均有CD56、CD4及CD123表达，3例有CD304表达。1例患者拒绝化疗，早期死亡。2例患者初始采用DA+VP方案治疗后达到完全缓解，其中1例复发后再次使用相同方案达到完全缓解。1例患者对减量的DA+VP化疗无反应，随后使用维奈克拉+阿扎胞苷达到完全缓解。
BPDCN患者的恶性细胞常浸润骨髓、脾脏及淋巴结，具有特殊表型，预后较差。治疗应兼顾髓系和淋巴系统。含BCL-2抑制剂等新药联合去甲基化药物的治疗值得尝试。
