母细胞性浆细胞样树突细胞肿瘤二例

Blastic plasmacytoid dendritic cell neoplasm: two cases report

例1,女,24岁,面部淤青色斑块5个月;例2,男,77岁,全身散发结节、斑块5个月,有"银屑病"病史18年。组织病理真皮内均可见弥漫性母细胞样细胞单一性浸润,与表皮有无浸润带形成;免疫组化均示CD123(+)、CD56(+)、CD4(+)、CD3(-)、CD20(-);诊断为母细胞性浆细胞样树突细胞肿瘤。例1于发病第16个月死亡;例2失访。

A 2g-year-old female presented with purple plaques on the face for 5 months. A 77- year-old male presented with nodule, plaques over the whole body for 5 months, and had a history of ＂psoriasis＂ for 18 years. Histologic examination showed a diffuse infiltrate composed of blastic lymphocytes and a grenz zone below the epidermis. Immunohistochemical staining showed positivity of CD123, CD56, CD4 and negativity of CD3 and CD20. The two cases were diagnosed as blastic plasmacytoid dendritic cell neoplasm. The case one only survived for 16 months while the case two lost follow-up.