title: Human BDCA2+CD123+CD56+ dendritic cells (DCs) related to blastic plasmacytoid dendritic cell neoplasm represent a unique myeloid DC subset.
abstract: Dendritic cells (DCs) comprise two functionally distinct subsets: plasmacytoid DCs (pDCs) and myeloid DCs (mDCs). pDCs are specialized in rapid and massive secretion of type I interferon (IFN-I) in response to nucleic acids through Toll like receptor (TLR)-7 or TLR-9. In this report, we characterized a CD56(+) DC population that express typical pDC markers including CD123 and BDCA2 but produce much less IFN-I comparing with pDCs. In addition, CD56(+) DCs cluster together with mDCs but not pDCs by genome-wide transcriptional profiling. Accordingly, CD56(+) DCs functionally resemble mDCs by producing IL-12 upon TLR4 stimulation and priming naïve T cells without prior activation. These data suggest that the CD56(+) DCs represent a novel mDC subset mixed with some pDC features. A CD4(+)CD56(+) hematological malignancy was classified as blastic plasmacytoid dendritic cell neoplasm (BPDCN) due to its expression of characteristic molecules of pDCs. However, we demonstrated that BPDCN is closer to CD56(+) DCs than pDCs by global gene-expression profiling. Thus, we propose that the CD4(+)CD56(+) neoplasm may be a tumor counterpart of CD56(+) mDCs but not pDCs.
authors.affiliation: Key Laboratory of Immunity and Infection, Institute of Biophysics, Chinese Academy of Sciences, Beijing, 100101 China.|Graduate School of the Chinese Academy of Sciences, Beijing, 100080 China.
authors.forename: Haisheng
authors.initials: H
authors.lastname: Yu
authors.affiliation: Key Laboratory of Protein and Peptide Pharmaceuticals, Institute of Biophysics, Chinese Academy of Sciences, Beijing, 100101 China.|Graduate School of the Chinese Academy of Sciences, Beijing, 100080 China.
authors.forename: Peng
authors.initials: P
authors.lastname: Zhang
authors.affiliation: Key Laboratory of Immunity and Infection, Institute of Biophysics, Chinese Academy of Sciences, Beijing, 100101 China.|Graduate School of the Chinese Academy of Sciences, Beijing, 100080 China.
authors.forename: Xiangyun
authors.initials: X
authors.lastname: Yin
authors.affiliation: Department of Cardiology, 306th Hospital of PLA, Beijing, 100101 China.
authors.forename: Zhao
authors.initials: Z
authors.lastname: Yin
authors.affiliation: Department of Cardiology, 306th Hospital of PLA, Beijing, 100101 China.
authors.forename: Quanxing
authors.initials: Q
authors.lastname: Shi
authors.affiliation: Key Laboratory of Protein and Peptide Pharmaceuticals, Institute of Biophysics, Chinese Academy of Sciences, Beijing, 100101 China.
authors.forename: Ya
authors.initials: Y
authors.lastname: Cui
authors.affiliation: Department of Gynecology and Obstetrics, Beijing Chaoyang Hospital, Capital Medical University, Beijing, 100020 China.
authors.forename: Guanyuan
authors.initials: G
authors.lastname: Liu
authors.affiliation: Department of Cardiology, 306th Hospital of PLA, Beijing, 100101 China.
authors.forename: Shouli
authors.initials: S
authors.lastname: Wang
authors.affiliation: Department of Experimental, Diagnostic, and Specialty Medicine, Hematopathology & Hematology Sections, Molecular Pathology Laboratory, S. Orsola-Malpighi Hospital, Bologna University, Bologna, 40126 Italy.
authors.forename: Pier Paolo
authors.initials: PP
authors.lastname: Piccaluga
authors.affiliation: Key Laboratory of Protein and Peptide Pharmaceuticals, Institute of Biophysics, Chinese Academy of Sciences, Beijing, 100101 China.
authors.forename: Taijiao
authors.initials: T
authors.lastname: Jiang
authors.affiliation: Key Laboratory of Immunity and Infection, Institute of Biophysics, Chinese Academy of Sciences, Beijing, 100101 China.
authors.forename: Liguo
authors.initials: L
authors.lastname: Zhang
mesh_terms: D015415:Biomarkers / Q000378:metabolism; D019002:CD56 Antigen / Q000235:genetics* / Q000276:immunology; D019070:Cell Lineage / Q000235:genetics / Q000276:immunology; D003713:Dendritic Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D015870:Gene Expression; D019337:Hematologic Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D006801:Humans; D016130:Immunophenotyping; D007370:Interferon Type I / Q000096:biosynthesis / Q000378:metabolism; D018664:Interleukin-12 / Q000096:biosynthesis / Q000378:metabolism; D053650:Interleukin-3 Receptor alpha Subunit / Q000235:genetics* / Q000276:immunology; D037181:Lectins, C-Type / Q000235:genetics* / Q000276:immunology; D008562:Membrane Glycoproteins / Q000235:genetics* / Q000276:immunology; D022423:Myeloid Cells / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D011971:Receptors, Immunologic / Q000235:genetics* / Q000276:immunology; D009626:Terminology as Topic; D051197:Toll-Like Receptor 4 / Q000235:genetics / Q000276:immunology; D051199:Toll-Like Receptor 7 / Q000235:genetics / Q000276:immunology; D051217:Toll-Like Receptor 9 / Q000235:genetics / Q000276:immunology
chemical_list: D015415:Biomarkers; D019002:CD56 Antigen; C443299:CLEC4C protein, human; C115022:IL3RA protein, human; D007370:Interferon Type I; D053650:Interleukin-3 Receptor alpha Subunit; D037181:Lectins, C-Type; D008562:Membrane Glycoproteins; C522918:NCAM1 protein, human; D011971:Receptors, Immunologic; C495345:TLR4 protein, human; C495348:TLR7 protein, human; C495371:TLR9 protein, human; D051197:Toll-Like Receptor 4; D051199:Toll-Like Receptor 7; D051217:Toll-Like Receptor 9; D018664:Interleukin-12