title: Blastic Plasmacytoid Dendritic Cell Neoplasm: From Origin of the Cell to Targeted Therapies.
abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy with an aggressive clinical course. It is grouped with acute myeloid leukemia-related precursor neoplasms in the 2008 World Health Organization classification. Most patients with BPDCN have skin lesions at diagnosis and subsequent or simultaneous involvement of the bone marrow, peripheral blood, and lymph nodes. Patients usually respond to initial chemotherapy but often relapse. Stem cell transplantation may improve survival. This neoplasm is derived from precursors of plasmacytoid dendritic cells and is characterized by the coexpression of the immunophenotypic markers CD4, CD56, CD123, blood dendritic cell antigen-2, blood dendritic cell antigen-4, CD2AP, and lineage(-). Atypical immunophenotype expression may be present, making diagnosis difficult. BPDCN is often associated with a complex karyotype, frequent deletions of tumor suppressor genes, and mutations affecting either the DNA methylation or chromatin remodeling pathways. A better understanding of the etiology and pathophysiology of this neoplasm could open the way to new therapies targeting specific signaling pathways or involving epigenetics.
authors.affiliation: Department of Hematology, Centre Hospitalier, Le Mans, France. Electronic address: klaribi@ch-lemans.fr.
authors.forename: Kamel
authors.initials: K
authors.lastname: Laribi
authors.affiliation: Department of Hematology, Centre Hospitalier, Le Mans, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Denizon
authors.affiliation: Department of Hematology, Centre Hospitalier, Le Mans, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Besançon
authors.affiliation: Department of Hematology, Centre Hospitalier, Le Mans, France.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Farhi
authors.affiliation: Laboratory of Hematology, Centre Hospitalier, Le Mans, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Lemaire
authors.affiliation: Laboratory of Anathomopathology, Centre Hospitalier, Le Mans, France.
authors.forename: Jeremy
authors.initials: J
authors.lastname: Sandrini
authors.affiliation: Clinical Research Center, Centre Hospitalier, Le Mans, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Truong
authors.affiliation: Department of Hematology, Centre Hospitalier, Le Mans, France.
authors.forename: Habib
authors.initials: H
authors.lastname: Ghnaya
authors.affiliation: Department of Medicine, Pôle Sante Sud, Le Mans, France.
authors.forename: Alix
authors.initials: A
authors.lastname: Baugier de Materre
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D001752:Blast Crisis / Q000473:pathology; D003713:Dendritic Cells / Q000473:pathology*; D019337:Hematologic Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology*; D006801:Humans; D016130:Immunophenotyping; D015470:Leukemia, Myeloid, Acute / Q000473:pathology; D012878:Skin Neoplasms; D033581:Stem Cell Transplantation
chemical_list: D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor
keywords: Blastic plasmacytoid dendritic cell neoplasm; Cytogenetics; Diagnosis; Epidemiology; Immunophenotypic; Treatment