title: [Analysis of one case of adolescent blastic plasmacytoid dendritic cell neoplasm].
abstract: This study was purposed to summarize the clinical characteristics and laboratorial data of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in pediatric patients in order to enhance understanding this disease in diagnosis and therapy. A rare case of BPDCN in children was enrolled in this study. The blood routine test, examination of bone marrow cell morphology, histopathology and immunophenotype of the skin lesions were performed and analysed, the single cell suspensions of the biopsied skin mass were detected by flow cytometry. The results showed that tumor cells expressed CD4, CD56, CD43 and CD123, while not expressed CD19, CD20, CD3, CD8, CD13, CD11b and myeloperoxidase (MPO). According to the clinical and laboratorial features and the results from histopathological and immunophenotype examinations, BPDCN was confirmed. It is concluded that BPDCN in children is an extremely rare hematopoietic malignancy with presenting a rapidly and fatally aggressive clinical course. The diagnosis of this disease is mainly based on the clinical presentations, pathologic and immunohistochemical features. BPDCN is a highly aggressive disease, its prognosis is very poor, its pathogenesis remans still unclear. A standard treatment protocol for BPDCN has not yet been established.
authors.affiliation: Department of Pediatrics, Sun Yet-Sen University, Guangzhou, Guangdong Province, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Ma
authors.forename: Yang
authors.initials: Y
authors.lastname: Li
authors.forename: Ling
authors.initials: L
authors.lastname: Liu
authors.forename: Hai-Xia
authors.initials: HX
authors.lastname: Guo
authors.forename: Hong-Man
authors.initials: HM
authors.lastname: Xue
authors.forename: Su-Xia
authors.initials: SX
authors.lastname: Lin
authors.forename: Hong-Gui
authors.initials: HG
authors.lastname: Xu
authors.forename: Shao-Liang
authors.initials: SL
authors.lastname: Huang
authors.forename: Chun
authors.initials: C
authors.lastname: Chen
authors.forename: Jian-Pei
authors.initials: JP
authors.lastname: Fang
mesh_terms: D000293:Adolescent; D003713:Dendritic Cells; D019337:Hematologic Neoplasms; D006801:Humans; D008297:Male; D012878:Skin Neoplasms; D008258:Waldenstrom Macroglobulinemia