儿童母细胞性浆细胞样树突细胞肿瘤1例分析

摘要本文分析1例儿童母细胞性浆细胞样树突细胞肿瘤（BPDCN）的诊断经验以提高对该病的认识。通过对该例儿童BPDCN病例进行了确诊分析，结合相关文献总结该疾病临床及实验室检查特点。对患儿皮肤肿物活检后行肿瘤细胞悬液流式细胞免疫荧光分析。结果表明，肿瘤细胞表达浆细胞样树突细胞标记CD123，同时表达CD4、CD56，不表达其它髓系、T细胞、B细胞特异性标记；根据临床资料、实验室检查、皮肤肿物病理及免疫组织化学检查结果，本例患者确诊为母细胞性浆细胞样树突细胞肿瘤。结论：儿童母细胞性浆细胞样树突细胞肿瘤非常罕见，诊断依赖于临床资料、组织病理学和免疫组织化学标记，该病病程呈侵袭性，预后差且发病机制在目前尚未明确。无标准治疗方案。

Analysis of One Case of Adolescent Blastic Plasmacytoid Dendritic Cell Neoplasm

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 rotine 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, CD11 b 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.