title: CD4+CD56+ hematodermic neoplasms bear a plasmacytoid dendritic cell phenotype.
abstract: CD4+CD56+ hematodermic neoplasms (HNs) with initial presentation in the skin are characterized by highly aggressive behavior and poor prognosis. Recent studies indicate that malignant cells, which are devoid of common T-, B-, NK-, and myeloid lineage markers, may be of plasmacytoid dendritic cell (pDC) origin. We undertook a study to assess the expression of several pDC-associated molecules on a series of 5 CD4+CD56+ HN cases. CD123 was expressed in all 5 cases, with some heterogeneity in individual cases. All but one case revealed fine membranous BDCA-2 staining of the dermal infiltrate. pDC-like phenotype of the malignant infiltrating cells was confirmed by costaining of BDCA-2+ cells with CD123 and CD4. MxA protein, representing the surrogate marker for lesional type I interferon activity, was expressed in 4 of 5 evaluated cases. Our findings further substantiate the putative pDC origin of CD4+CD56+ HNs.
authors.affiliation: Department of Dermatology, University Hospital Zurich, Zurich 8091, Switzerland. mirjana.urosevic@usz.ch
authors.forename: Mirjana
authors.initials: M
authors.lastname: Urosevic
authors.forename: Curdin
authors.initials: C
authors.lastname: Conrad
authors.forename: Jivko
authors.initials: J
authors.lastname: Kamarashev
authors.forename: Kenji
authors.initials: K
authors.lastname: Asagoe
authors.forename: Antonio
authors.initials: A
authors.lastname: Cozzio
authors.forename: Günter
authors.initials: G
authors.lastname: Burg
authors.forename: Reinhard
authors.initials: R
authors.lastname: Dummer
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D015704:CD4 Antigens / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D019070:Cell Lineage; D003713:Dendritic Cells / Q000276:immunology / Q000473:pathology*; D005260:Female; D019204:GTP-Binding Proteins / Q000032:analysis; D006801:Humans; D016130:Immunophenotyping; D053650:Interleukin-3 Receptor alpha Subunit; D037181:Lectins, C-Type / Q000032:analysis; D008223:Lymphoma / Q000276:immunology / Q000473:pathology*; D008297:Male; D008562:Membrane Glycoproteins / Q000032:analysis; D008875:Middle Aged; D064547:Myxovirus Resistance Proteins; D011971:Receptors, Immunologic / Q000032:analysis; D016185:Receptors, Interleukin-3 / Q000032:analysis; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; D015704:CD4 Antigens; D019002:CD56 Antigen; C443299:CLEC4C protein, human; C115022:IL3RA protein, human; D053650:Interleukin-3 Receptor alpha Subunit; D037181:Lectins, C-Type; C579071:MX1 protein, human; D008562:Membrane Glycoproteins; D064547:Myxovirus Resistance Proteins; D011971:Receptors, Immunologic; D016185:Receptors, Interleukin-3; D019204:GTP-Binding Proteins