母细胞性浆细胞样树突细胞肿瘤的临床病理学特征分析

Clinicopathological characteristics of blastic plasmacytoid dendritic cell neoplasm

目的 加深对母细胞性浆细胞样树突细胞肿瘤（BPDCN）的认识.方法 回顾性分析中国医学科学院北京协和医学院肿瘤医院病理科会诊诊断的2例BPDCN的临床病理学特点,并结合文献进行讨论.结果 2例患者均为老年男性,均以皮肤结节为临床表现,形态学显示肿瘤细胞弥漫浸润真皮全层,均可见与表皮间的无细胞带.肿瘤细胞小至中等大小,核呈圆形或卵圆形,不规则,可见小核仁,核分裂象多见.免疫组织化学染色结果显示2例CD4及CD56均阳性,仅1例CD123呈阳性,CD20及CD3均阴性.结论 BPDCN是一种罕见的淋巴造血系统恶性肿瘤,易累及皮肤,形态学及特征性的免疫表型是正确诊断BPDCN的关键.

Objective To improve the understanding of blastic plasmacytoid dendric cell neoplasm （BPDCN）. Methods The clinicopathological features of 2 cases with BPDCN diagnosed in the Department of Pathology in Cancer Hospital of Chinese Academy of Medical Sciences and Peking Union Medical College were analyzed retrospectively. The relevant literatures were reviewed as well. Results The two BPDCN patients were both elderly men with skin nodules. The tumor cells infiltrated diffusely into the whole dermis with sparing the epidermis. BPDCN cells were small to medium size with round or oval irregular nuclei and small nucleoli. Numerous mitoses figures were seen. Immunohistochemistry stain showed that CD4 and CD56 were positive and CD20 and CD3 were negative in the 2 BPDCN cases. Only 1 case was positive for CD123. Conclusions BPDCN is a rare malignant hematopoietic tumor, which is likely to involve skin. The features of morphology and immunophenotype are crucial to make correct diagnosis.