To date, the research on dendritic cells (DCs) and their correlated neoplasms has not been clear. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) and mature plasmacytoid dendritic cell proliferation (MPDCP) are two types of malignancies originating from plasmacytoid dendritic cells (pDCs). Some evidence has indicated the existence of other pDC neoplasms. In addition, cases of myeloid neoplasms (MNs), acute myeloblastic leukemia (AML), and myelodysplastic syndrome (MDS) with increased pDCs (AML/MDS-pDCs) seem to have immature DCs according to the vaguely consistent expression of markers among MNs and pDCs, which appear to fit the developmental pattern of normal DCs. We analyzed 14 AML/MDS-pDC cases mainly for their immunophenotype by flow cytometry and inferred their CD expression pattern. The patients' clinical information and other laboratory data were collected and reviewed. AML/MDS-pDCs show a different pattern of markers from BPDCN and MPDCP. Three maturation-involved stages were found in these AML/MDS-pDCs patients. Stage I was the most immature stage and displayed an expression profile of CD34+/st+ CD117+/st+ BDCA2- BDCA4- CD123+ HLA-DR+/st+ CD4- CD45dim+ ; Stage II was the more immature stage displayed a phenotype of CD34dim+ CD117dim+ BDCA2-/dim+ BDCA4-/dim+ CD123st+ HLA-DR+/st+ CD4- CD45+ ; and Stage III was the mature stage showed CD34- CD117- BDCA2+ /BDCA4+ CD123st+ HLA-DR+/st+ CD4+ CD45+/st+ . Three maturation-involved stages overlapped well with the phenotypes of normal DC progenitors in a continuously developmental process: granulocyte, monocyte, and DC progenitors (GMDPs) and/or monocyte and DC progenitors (MDPs), common DC progenitors (CDPs), pDCs, and/or pre-DCs. In this study, we considered AML/MDS-pDCs as entities that were distinct from BPDCN and MPDCP and correlated the components of this tumor with the normal DC differentiation pathway, which provides new evidence for understanding DC neoplasms. © 2019 International Society for Advancement of Cytometry.

中文翻译：

---

浆细胞样树突状细胞分化程度升高的骨髓肿瘤反映了树突状细胞的成熟过程。

迄今为止，关于树突状细胞（DC）及其相关肿瘤的研究还不清楚。弹性浆细胞样树突状细胞瘤（BPDCN）和成熟浆细胞样树突状细胞增殖（MPDCP）是源自浆细胞样树突状细胞（pDC）的两种恶性肿瘤。一些证据表明存在其他pDC肿瘤。此外，根据MNs和MNs之间标记物的一致表达，髓样肿瘤（MNs），急性粒细胞性白血病（AML）和骨髓增生异常综合征（MDS）的pDC（AML / MDS-pDCs）升高的病例似乎具有不成熟的DC。 pDC，似乎适合正常DC的发育模式。我们通过流式细胞术分析了14例AML / MDS-pDC病例的主要免疫表型，并推断了其CD表达模式。患者的 收集并审查了临床信息和其他实验室数据。AML / MDS-pDC与BPDCN和MPDCP显示出不同的标记模式。在这些AML / MDS-pDCs患者中发现了三个成熟阶段。第一阶段是最不成熟的阶段，显示了CD34 + / st + CD117 + / st + BDCA2- BDCA4- CD123 + HLA-DR + / st + CD4- CD45dim +的表达谱；II期是较不成熟的CD34dim + CD117dim + BDCA2- / dim + BDCA4- / dim + CD123st + HLA-DR + / st + CD4- CD45 +的表型; 而阶段III是成熟阶段，显示CD34- CD117- BDCA2 + / BDCA4 + CD123st + HLA-DR + / st + CD4 + CD45 + / st +。在一个持续发展的过程中，三个成熟阶段与正常DC祖细胞的表型重叠良好：粒细胞，单核细胞和DC祖细胞（GMDP）和/或单核细胞和DC祖细胞（MDP），常见的DC祖细胞（CDP），pDC和/或pre-DC。在这项研究中，我们将AML / MDS-pDCs视为不同于BPDCN和MPDCP的实体，并将该肿瘤的成分与正常的DC分化途径相关联，这为了解DC肿瘤提供了新的证据。©2019国际细胞计数学会。