Purpose of Review

The purpose of this review was to summarize the clinical, diagnostic, and therapeutic features of blastic plasmacytoid dendritic cell neoplasm (BPDCN).

Recent Findings

Several case reports and series revealed new clinical, molecular, diagnostic, and therapeutic aspects of the disease. The clinical presentation diversity has been confirmed, with frequent leukemic non-cutaneous or rare atypical manifestations. The clonal evolution in the development of BPDCN has not been sufficiently elucidated. Although certain immunophenotypic markers (CD4, TCL1, CD123, CD56, CD303) are indicative of BPDCN, the diagnosis remains in certain cases challenging. Adult (ALL)-type chemotherapy followed by hematopoietic stem cell transplantation (HSCT) is related to a favorable outcome, while chemotherapy alone seems enough in children. Future studies should continue to investigate whether CD123-directed therapies could be utilized.

Summary

BPDCN is a rare aggressive malignancy that needs an aggressive induction therapy. Although a diagnostic consensus is still lacking, and large retrospective studies are also needed to obtain standardized treatment guidelines, the future perspectives are encouraging, because of novel therapeutic agents that are under investigation.

中文翻译：

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树突状细胞白血病：综述。

审查目的

本综述的目的是总结母细胞性浆细胞样树突细胞肿瘤 (BPDCN) 的临床、诊断和治疗特征。

最近的发现

一些病例报告和系列报道揭示了该疾病的新临床、分子、诊断和治疗方面。临床表现的多样性已得到证实，常见的白血病非皮肤或罕见的非典型表现。BPDCN 发展过程中的克隆进化尚未得到充分阐明。尽管某些免疫表型标志物（CD4、TCL1、CD123、CD56、CD303）可指示 BPDCN，但在某些情况下诊断仍然具有挑战性。成人 (ALL) 型化疗后进行造血干细胞移植 (HSCT) 与良好的结果相关，而仅对儿童进行化疗似乎就足够了。未来的研究应继续调查是否可以利用 CD123 导向疗法。

概括

BPDCN 是一种罕见的侵袭性恶性肿瘤，需要积极的诱导治疗。尽管仍缺乏诊断共识，并且还需要大型回顾性研究来获得标准化的治疗指南，但由于正在研究新的治疗药物，未来的前景令人鼓舞。