Sang Wei, Wang Chao-fu, Cheng Yu-fan, Liu Xia, Li Xin-xia, Gulinar Abulajiang, Li Jun-zhi, Zhang Wei
Department of Pathology, Xinjiang Medical University First Hospital, Urumqi 830054, China.
Zhonghua Bing Li Xue Za Zhi. 2012 May;41(5):326-30. doi: 10.3760/cma.j.issn.0529-5807.2012.05.009.
To study the clinicopathologic features and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm.
The clinical, morphology and immunophenotypic features were analyzed in 3 cases of blastic plasmacytoid dendritic cell neoplasm, with review of literature.
The pathologic changes of these tumors accorded with that of blastic plasmacytoid dendritic cell neoplasm, and they also had new characteristics, including lineage other than T, B, myeloid and NK cells, and immunophenotypes of CD56(+) CD4(-) CD123(+) TdT(+) CD43(+) CD68(+) , CD56(+) CD4(+) CD123(-) TdT(+) CD43(+) CD68(-) and CD56(+) CD4(+) CD123(-/+) TdT(-) CD43(+) CD68(+) in the 3 cases, respectively. Bone marrow involvement was found 5 years later in case 1, and was then stable after chemotherapy; case 2 and case 3 were died 5 and 2 months after diagnosis, respectively.
Blastic plasmacytoid dendritic cell neoplasm is a heterogeneous group of lymphoproliferative disorders, with different clinical, morphologic and immunophenotypic features.
研究母细胞性浆细胞样树突状细胞肿瘤的临床病理特征及鉴别诊断。
分析3例母细胞性浆细胞样树突状细胞肿瘤的临床、形态学及免疫表型特征，并复习相关文献。
这些肿瘤的病理改变符合母细胞性浆细胞样树突状细胞肿瘤，且具有新的特点，包括T、B、髓系及NK细胞以外的谱系，3例的免疫表型分别为CD56(+) CD4(-) CD123(+) TdT(+) CD43(+) CD68(+)、CD56(+) CD4(+) CD123(-) TdT(+) CD43(+) CD68(-)和CD56(+) CD4(+) CD123(-/+) TdT(-) CD43(+) CD68(+)。病例1在5年后发现骨髓受累，化疗后病情稳定；病例2和病例3分别在诊断后5个月和2个月死亡。
母细胞性浆细胞样树突状细胞肿瘤是一组异质性的淋巴增殖性疾病，具有不同的临床、形态学及免疫表型特征。
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