In this review, we aim to provide a summary of the diverse immunophenotypic presentations of distinct entities associated with plasmacytoid dendritic cell (pDC) proliferation. These entities include the following: (1) blastic plasmacytoid dendritic cell neoplasm (BPDCN); (2) mature pDC proliferation (MPDCP), most commonly seen in chronic myelomonocytic leukemia (CMML); and (3) myeloid neoplasms with pDC differentiation, in which pDCs show a spectrum of maturation from early immature pDCs to mature forms, most commonly seen in acute myeloid leukemia (pDC-AML). Our aim is to provide a flow cytometry diagnostic approach to these distinct and sometimes challenging entities and to clarify the immunophenotypic spectrum of neoplastic pDCs in different disease presentations. In this review, we also cover the strategies in the evaluation of residual disease, as well as the challenges and pitfalls we face in the setting of immune and targeted therapy. The differential diagnosis will also be discussed, as blasts in some AML cases can have a pDC-like immunophenotype, mimicking pDCs.
本综述旨在总结与浆细胞样树突状细胞(pDC)增殖相关的不同疾病实体的多样化免疫表型特征。这些疾病实体包括:(1)原始浆细胞样树突状细胞肿瘤(BPDCN);(2)成熟pDC增殖(MPDCP),最常见于慢性粒单核细胞白血病(CMML);(3)伴有pDC分化的髓系肿瘤,其中pDC呈现从早期未成熟到成熟形态的连续分化谱系,最常见于急性髓系白血病(pDC-AML)。本文旨在为这些独特且有时具有诊断挑战性的疾病实体提供流式细胞术诊断路径,并阐明不同疾病表现中肿瘤性pDC的免疫表型谱。此外,本综述还涵盖微小残留病评估策略,以及在免疫治疗和靶向治疗背景下所面临的挑战与误区。鉴别诊断亦将予以讨论,因为部分AML病例中的原始细胞可能呈现类似pDC的免疫表型,从而与pDC相混淆。
Flow Cytometry Profiling of Plasmacytoid Dendritic Cell Neoplasms