Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic study of two cases

LI Liang; SUN Xiao-feng; HAN Cui-hong; ZHOU Feng-juan

doi:10.3969/j.issn.1007-8096.2017.05.006

Abstract：Objective To explore the clinicopathologic features,immunohistochemical features,diagnosis and differential diagnosis of blastic plasmacytoid dendritic cell neoplasms (BPDCN).Methods The clinical,histopathological and immunohistochemical features of two cases of BPDCN were analyzed and the literatures were reviewed.Results Both patients were males with age of 42 and 72 years respectively.Both patients presented with multiple skin patches.Microscopically,tumor cells were diffuse infitration in the dermis.Tumor cells were medium to large in size,irregular nuclei,dispersed nuclear chromatin,indistinct nucleolus and scant cytoplasm.Immunohistochemical staining showed that two cases were positive expression of CD4,CD56,CD43 and CD123,and negative expression of CD117,MPO,CD68,PAX5,CD20,CD3 and CD5;TDT was focal positive in one case.Conclusions BPDCN is a rare and highly aggressive neoplasm of haematopoietic and lymphoid tissues.Except for the skin,bone marrow and lymph node can also be involved.The tumor cells express CD4,CD56,CD43,CD123 and lack the other lineage specific markers.It should be distinguished from acute leukaemia and lymphoma.It is easily misdiagnosed as other types of lymphoma and acute leukemia because of its extremely rare,but we can make the correct diagnosis according to its clinicopathologic features and immunohistochemical results.