母细胞性浆细胞样树突细胞肿瘤5例临床病理分析

Clinicopathological characteristics of blastic plasmacytoid dendritic cell neoplasm

目的探讨母细胞性浆细胞样树突细胞肿瘤的临床病理学特征。方法应用光镜及免疫组化法观察5例母细胞性浆细胞样树突细胞肿瘤的组织学特点及免疫表型,并复习相关文献。结果 5例中3例为男性,2例为女性,年龄分别为53、72、40、14及15岁。5例病变均以皮肤斑块或结节为首发表现,并均累及骨髓,其中1例累及淋巴结。皮肤病变显示肿瘤细胞在真皮弥漫分布,不累及表皮。肿瘤细胞中等大小,核圆形或椭圆形,染色质细腻。5例均表达CD56、CD123、CD43,3例表达CD4;5例均不表达CD8、Td T及EBER。5例均行化疗,1例死亡,2例放弃治疗后失访,2例健在。结论母细胞性浆细胞样树突细胞肿瘤是一种罕见的肿瘤,具有独特的临床病理特点,诊断依靠病理组织学及部分辅助方法。

Purpose To explore the clinicopathological characteristics of blastic plasmacytoid dendritic cell neoplasms. Methods The histopathological features and immunophenotype of four cases of blastic plasmacytoid dendritic cell neoplasms were studied, and relevant literature was reviewed. Results Among the five cases, three were males and two were females. The ages of the patients were 53,72, 40, 14, and 15 years, respectively. The initial symptoms for the five patients were skin macules or nodules, all had bone mar- row involvement, with one case involving lymph nodes. Microscopically, the tumor ceils were disseminated in the dermis, without epi- dermal involvement. The tumor ceils were of medium size with round or oval nucleus and fine chromatin. All five cases expressed CD56, CD123, and CD43. three expressed CIM. CD8, TdT, and EBER were negative in all five cases. All five patients received chemotherapy. One patient died; two withdrawn therapy and was lost to follow-up. The other two patients are currently alive. Conclu- sion Blastic plasmacytoid dendritic cell neoplasm is a rare tumor featuring unique clinicopathological characteristics. And the diagno- sis relies on histopathological analysis and some ancillary markers.