Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic study of 7 cases

Abstract

To improve the clinicopathological understanding of blastic plasmacytoid dendritic cell neoplasm (BPDCN). A total of 7 BPDCN patients were recruited from 2009 to 2013. And their clinicopathological and immunohistological features, treatment and prognosis were retrospectively analyzed. There were 6 males and 1 female. The median age range was 60 (6-72) years at the time of diagnosis. The skin lesion at the time of diagnosis presented as generalized (n = 6) or solitary (n = 1) plaques or nodules. Histologic examination showed a diffuse or nodular infiltrate composed of intermediate-sized cells in dermis sparing epidermis area. The tumor cells contained round to ovoid nucleus, finely dispersed chromatin and 1 or 2 small-sized nucleoli. Most neoplastic cells were positive for CD4, CD56 and CD123 while negative for CD3, CD20, CD30, CD34, CD79a, MPO and EBER. Two patients received chemotherapy.One had no response while another relapsed rapidly. Two patients had a spontaneous regression of skin lesions and one died 2 years later. And no systematic infiltration was detected at diagnosis. BPDCN is a rare hematopoietic malignancy with specific immunophenotypes.It primarily affects elderly males and frequently presents initially as cutaneous involvement.It may be easily misdiagnosed and has a rapid course and a poor prognosis.