Blastic Plasmacytoid Dendritic Cell Neoplasm: Progress in Cell Origin, Molecular Biology, Diagnostic Criteria and Therapeutic Approaches
Blastic Plasmacytoid Dendritic Cell Neoplasm: Progress in Cell Origin, Molecular Biology, Diagnostic Criteria and Therapeutic Approaches
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母细胞性浆细胞样树突状细胞肿瘤：细胞起源、分子生物学、诊断标准和治疗方法的进展
DOI：
10.1007/s11596-021-2393-3
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发表时间：
2021-07-03
影响因子：
2.4
通讯作者：
Yu, Li
中科院分区：
文献类型：
--
作者：
Cheng, Wei;Yu, Tian-tian;Yu, Li
母浆细胞样树突状细胞肿瘤（BPDCN）是一种罕见的血液系统恶性肿瘤，其特征是复发性皮肤结节，具有侵袭性的临床病程，可迅速累及血液系统器官，预后差，总生存率低。BPDCN来源于浆细胞样树突状细胞（pDCs），其发病机制尚不清楚。肿瘤细胞CD4、CD56、白细胞介素-3受体α链（CD123）、血树突状细胞抗原2 （BDCA 2/CD303）、血树突状细胞抗原4 （BDCA4）和转录因子（E蛋白）E2-2 （TCF4）表达异常。最好的治疗药物是根据经验采用那些用于白血病或淋巴瘤的药物。伴有耐药的复发通常发生得很快。首次完全缓解后推荐进行干细胞移植，而tagraxofusp是第一个靶向治疗。本文就BPDCN的细胞来源、与正常pdcn的联系、临床特征、基因突变及治疗进展等方面进行综述。本文综述了BPDCN的机制和新的治疗方法。
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy characterized by recurrent skin nodules, an aggressive clinical course with rapid involvement of hematological organs, and a poor prognosis with poor overall survival. BPDCN is derived from plasmacytoid dendritic cells (pDCs) and its pathogenesis is unclear. The tumor cells show aberrant expression of CD4, CD56, interleukin-3 receptor alpha chain (CD123), blood dendritic cell antigen 2 (BDCA 2/CD303), blood dendritic cell antigen 4 (BDCA4) and transcription factor (E protein) E2-2 (TCF4). The best treatment drugs are based on experience by adopting those used for either leukemia or lymphoma. Relapse with drug resistance generally occurs quickly. Stem cell transplantation after the first complete remission is recommended and tagraxofusp is the first targeted therapy. In this review, we summarize the differentiation of BPDCN from its cell origin, its connection with normal pDCs, clinical characteristics, genetic mutations and advances in treatment of BPDCN. This review provides insights into the mechanisms of and new therapeutic approaches for BPDCN.