title: Blastic plasmacytoid dendritic cell neoplasm: A case report.
abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as agranular cluster of differentiation (CD)4+/CD56+hematodermic neoplasm, is a rare and aggressive type of lymphoma, with only ~100 cases reported worldwide. BPDCN is a hematological malignancy derived from precursors of plasmacytoid dendritic cells and is clinically characterized by cutaneous manifestations involving the lymph nodes and peripheral blood, a leukemia-like dissemination and a poor prognosis. The present study reports the case of a 54-year-old male who presented with symptoms characteristic of BPDCN. Pathological and immunohistochemical analysis of abdominal skin lesion biopsies were used to determine a diagnosis of stage IIIE BPDCN. Although cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy was administered, the patient succumbed to BPDCN nine days after the discontinuation of chemotherapy. Thus, the period from BPDCN presentation to mortality was ≤3 months. The case reported in the present study was characterized by rapid development and poor prognosis, and displayed additional features of BPDCN, including systemic dissemination and a short survival period.
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Wei
authors: W
authors: Wang
authors: Department of Pathology, Third Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Wensheng
authors: W
authors: Li
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Jin-Jing
authors: JJ
authors: Jia
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Yan
authors: Y
authors: Zheng
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Hao
authors: H
authors: Wang
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Xiao-Min
authors: XM
authors: Gao
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Xin-Yu
authors: XY
authors: Dong
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Qiong
authors: Q
authors: Tian
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Hui-Ling
authors: HL
authors: Jing
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Xin
authors: X
authors: Wang
authors: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, P.R. China.
authors: Sheng-Xiang
authors: SX
authors: Xiao
keywords: blastic plasmacytoid dendritic cell neoplasm; cyclophosphamide; diagnosis; doxorubicin; prognosis; treatment; vincristine and prednisolone chemotherapy