Analysis of one case of adolescent blastic plasmacytoid dendritic cell neoplasm / 中国实验血液学杂志
Journal of Experimental Hematology
;
(6): 140-145, 2013.
Artigo
em Chinês
| WPRIM
| ID: wpr-325196
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Neoplasias Cutâneas
/
Células Dendríticas
/
Macroglobulinemia de Waldenstrom
/
Neoplasias Hematológicas
Tipo de estudo:
Guia de Prática Clínica
/
Estudo prognóstico
Limite:
Adolescente
/
Humanos
/
Masculino
Idioma:
Chinês
Revista:
Journal of Experimental Hematology
Ano de publicação:
2013
Tipo de documento:
Artigo
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