母细胞性浆细胞样树突细胞肿瘤

Blastic plasmacytoid dendritic cell neoplasm

报告1例母细胞性浆细胞样树突细胞肿瘤。患者女,26岁。面部结节、斑块半年。皮肤科检查:右颧部可见约4 cm×6 cm暗红色浸润性斑块,类圆形分叶状,境界清楚,皮损中央破溃。皮损组织病理检查:真皮全层及皮下脂肪间见较多致密的异形淋巴细胞浸润,细胞中等大小,核膜薄,染色质细,核分裂象易见。免疫组化:CD4(+)、CD56(+)、CD123(+)、CD43(+)、CD34(+),Ki-67(>60%阳性);CD20、CD79a、丝氨酸蛋白酶(granzyme)B、CD3、CD45RO、CD68、末端脱氧核苷酸转移酶(TDT)、髓过氧化物酶(MPO)、T细胞内抗原(TIA)-1均阴性。诊断:母细胞性浆细胞样树突细胞肿瘤。

A case of blastic plasmacytoid dendritic cell neoplasm （BPDCN） is reported. A 26-year-old female presented with nodule and plaque on the face for 6 months. Physical examination revealed a demarcated, dark erythematous and infiltrated plaque, sized 4cm × 6 cm in diameter, on the right cheek. Histopathological examination showed diffuse infiltrate of numerous medium-sized atypical lymphocytes with irregular nuclei in superficial dermis and subcutaneous fat tissue. The biastic cells showed fine-granular chromatin and abundant mitotic figures. Immunohistoehemical staining showed that tumor cells were positive for CD4, CD56,CD123,CD34 and CD43, but negative for CD3, CD45RO, CD68, TDT, CD20, CD79A, granzyme B, TIA-1 and MPO. The diagnosis of BPDCN was made.