RESUMO
Purpose To study the clinicopathological characteristics of blastic Plasmacytoid dendritic cell neoplasms, and improve the acknowledgement and diagnostic level. Methods The clinicopathological features, immunohistochemistry and EBV test results of 10 cases with BPDCN were analyzed retrospectively, with review of literature. Results Skin lesions were the first symptom in 10 patients, tumor cells expression of CD43, CD56, CD4 was positive in 8 cases, CD123 were positive in 5 cases, the T and B cell specific markers is not expression. EB virus encoded small RNA(EBER ) in situ hybridization is negative;no clonal rearrangement of B cell receptor(BCR) or T cell receptor(TCR) gene. Conclusion BPDCN is a rare malignant tumor of the skin, easy to involve the skin. It is necessary to combine clinical with pathological for definite diagnosis.