Division of Diagnostic Haematopathology, European Institute of Oncology, IRCCS, Via Ripamonti 435, Milan 20141, Italy.
Division of Diagnostic Haematopathology, European Institute of Oncology, IRCCS, Via Ripamonti 435, Milan 20141, Italy.
Hematol Oncol Clin North Am. 2020 Jun;34(3):511-521. doi: 10.1016/j.hoc.2020.01.002. Epub 2020 Mar 4.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic neoplasm with a dismal prognosis and no standard therapy. In the past, its cellular ontogenesis was obscure, and BPDCN had been erroneously named CD56/TdT blastic NK cell tumor and CD4/CD56 hematodermic neoplasm. Finally, in 2008, the BPDCN was correctly recognized as a neoplasm deriving from the malignant transformation of plasmacytoid dendritic cell precursors and classified among the myeloid neoplasms. Since then, the understanding of BPDCN biology has improved rapidly: the DNA mutational status of BPDCN has been extensively investigated revealing a spectrum perfectly resembling its myeloid lineage derivation.
原始滤泡性淋巴瘤（BPDCN）是一种罕见的血液系统恶性肿瘤，预后不良，目前尚无标准治疗方法。过去，其细胞发生学不明确，BPDCN 曾被错误地命名为 CD56/TdT 原始 NK 细胞肿瘤和 CD4/CD56 造血组织肿瘤。最终，在 2008 年，BPDCN 被正确地认定为一种源自浆细胞样树突状细胞前体的恶性转化的肿瘤，并被归类为髓系肿瘤。从那时起，BPDCN 的生物学特性得到了快速的认识：BPDCN 的 DNA 突变状态已经得到了广泛的研究，揭示了一个与髓系来源完全相似的谱。
