RESUMO
OBJECTIVE@#To analyze the clinical characteristics, pathological features, treatment response and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN), so as to provide reference for reducing clinical misdiagnosis and treatment regimens.@*METHODS@#The clinical, pathological features, treatment regimens and treatment response of 9 patients with BPDCN diagnosed and treated from January 1, 2013 to June 30,2019 in Xiangya Second Hospital of Central South University were analyzed respectively.@*RESULTS@#The most common site of involvement in 9 patients with BPDCN was skin; the pathological features were involvement of dermis, and the highest positive immunohistochemical indexes rate were CD4, CD43, LCA, CD123, and VIM (all 100%), followed by CD56 and CD68, TDT, CD5, PAX-5, CD3, CD8, CD20; MPO and EBER of 9 patients were negative. However, Ki-67 of the 9 patients showed a medican of 77. 5% (40%-90%), which suggests that CD4, CD43, LCA, CD123, and VIM were the most sensitive antigens for diagnosing BPDCN. Of the 9 patients, 2 cases were lost to follow-up, 2 cases were untreated, and 5 cases were treated, in which 2 cases received treatment of regiment for acute lymphoblastic leukemia and 3 cases received treatment of regimen for lymphoma. The PR of clinical symptoms in the first cycle in 5 patients were 100%, and 1 patient has survived for 6 years since the disease was diagnosed.@*CONCLUSION@#BPDCN is a rare and highly invasive lymphoid hematopoietic malignancy. The most common initial symptom of the patients is skin lesions. The most sensitive pathological indicators are CD4, CD43, LCA, VIM, and the BPDCN patients received treatment of regimens for lymphoid tumor show a better response rate.