文章：

母细胞性浆细胞样树突状细胞肿瘤（BPDCN）中树突状细胞非激活状态的特征及免疫缺陷

Features of non-activation dendritic state and immune deficiency in blastic plasmacytoid dendritic cell neoplasm (BPDCN)

原文发布日期：2019-12-06

DOI: 10.1038/s41408-019-0262-0

类型: Article

开放获取: 是

英文摘要：

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, male-predominant hematologic malignancy with poor outcomes and with just one recently approved agent (tagraxofusp). It is characterized by the abnormal proliferation of precursor plasmacytoid dendritic cells (pDCs) with morphologic and molecular similarities to acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS)/chronic myelomonocytic leukemia (CMML) in its presentation within the bone marrow and peripheral blood. To identify disease-specific molecular features of BPDCN, we profiled the bone marrow, peripheral blood, and serum samples from primary patient samples using an in-house hematologic malignancy panel (“T300” panel), transcriptome microarray, and serum multiplex immunoassays. TET2 mutations (5/8, 63%) were the most prevalent in our cohort. Using the transcriptome microarray, genes specific to pDCs (LAMP5, CCDC50) were more highly expressed in BPDCN than in AML specimens. Finally, the serum cytokine profile analysis showed significantly elevated levels of eosinophil chemoattractants eotaxin and RANTES in BPDCN as compared with AML. Along with the high levels of PTPRS and dendritic nature of the tumor cells, these findings suggest a possible pre-inflammatory context of this disease, in which BPDCN features nonactivated pDCs.
 

摘要翻译： 

母细胞性浆细胞样树突状细胞肿瘤（BPDCN）是一种罕见的、男性多发的血液系统恶性肿瘤，预后较差，且近期仅有一种获批药物（他拉罗福司）。该疾病的特征在于前体浆细胞样树突状细胞（pDC）的异常增殖，在骨髓和外周血中的表现具有与急性髓系白血病（AML）和骨髓增生异常综合征（MDS）/慢性粒单核细胞白血病（CMML）相似的形态学和分子特征。为了识别BPDCN的疾病特异性分子特征，我们使用内部血液系统恶性肿瘤检测 panel（“T300” panel）、转录组微阵列和血清多重免疫分析法，对原代患者样本的骨髓、外周血和血清样本进行了分析。TET2突变（5/8，63%）在我们的队列中最为普遍。通过转录组微阵列分析，pDC特异性基因（LAMP5、CCDC50）在BPDCN中的表达高于AML标本。最后，血清细胞因子谱分析显示，与AML相比，BPDCN中嗜酸性粒细胞趋化因子嗜酸细胞活化趋化因子和RANTES的水平显著升高。结合PTPRS的高水平表达和肿瘤细胞的树突状特性，这些发现提示该疾病可能存在一种前炎症背景，其中BPDCN以未活化的pDC为特征。

原文链接：

Features of non-activation dendritic state and immune deficiency in blastic plasmacytoid dendritic cell neoplasm (BPDCN)