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目的探讨母细胞性浆细胞样树突状细胞肿瘤(BPDCN)的临床病理特点、免疫组化、诊断与鉴别诊断。方法观察2例BPDCN的临床表现、组织学特征及免疫组化,并结合文献复习。结果 2例均为男性,年龄分别为42岁、72岁,均以多发性皮肤斑块就诊。组织形态学瘤细胞弥漫分布于真皮层,核形不规整、呈多形性,中等偏大、染色质细腻、核仁不明显,胞质稀少。免疫组化:2例肿瘤细胞均表达CD4、CD56、CD43和CD123,其中1例部分表达TDT,2例均不表达CD34、CD117、MPO、CD68、PAX5、CD20、CD3和CD5。结论 BPDCN是一种临床上呈高度侵袭性淋巴造血系统恶性肿瘤,最常见侵犯皮肤,骨髓和淋巴结也可以受累,因其极为罕见,容易误诊为其他类型的淋巴瘤及急性白血病,但只要充分认识其组织形态学与免疫组化特征,还是可以对其作出正确诊断。
Objective To investigate the clinicopathological characteristics, immunohistochemistry, diagnosis and differential diagnosis of bcllc cytoplasmic dendritic cell tumor (BPDCN). Methods The clinical manifestations, histological features and immunohistochemistry of 2 cases of BPDCN were observed and reviewed. Results Both cases were male, with the age of 42 years and 72 years old, respectively, with multiple skin plaques. Histomorphology Tumor cells diffusely distributed in the dermis, irregular nuclear shape, was pleomorphic, medium to large, chromatin delicate, not obvious nucleolus, scarce cytoplasm. Immunohistochemistry: CD4, CD56, CD43 and CD123 were expressed in both of the tumor cells. One of them expressed partial TDT, while none of the two expressed CD34, CD117, MPO, CD68, PAX5, CD20, CD3 and CD5. Conclusions BPDCN is a clinically highly malignant lymphoid hematopoietic system. The most common malignant tumor of the hematopoietic system is bone marrow and lymph nodes. Because it is extremely rare and easily misdiagnosed as other types of lymphoma and acute leukemia, Its histomorphology and immunohistochemical features, or can make the correct diagnosis of it.