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.
IntroductionBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly invasive type of malignant hematopoietic and lymphoid tissue tumor (1). Since BPDCN was initially reported by Adachi et al (2) in 1994, it has been successively reported in the literature. In 2004, Chaperot et al (3) identified that the BPDCN tumor functions similarly to plasmacytoid dendritic cells (pDC), and, thus, proposed that it may be derived from the precursor of the pDC. Subsequent studies determined that the BPDCN tumor cells express the highly specific pDC markers, blood dendritic cell antigen (BDCA)-2/cluster of differentiation (CD)303 and BDCA-4/CD304, supporting the hypothesis that the BPDCN tumor is derived from pDC (4). In 2005, the World Health Organisation (WHO) European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas recommended the use of the term CD4 + /CD56 + hematodermic neoplasm (5,6). In 2008, the tumor was officially named BPDCN in the WHO classification of lymphoid and hematopoietic tumors and was listed as a novel, independent type of hematopoietic and lymphoid tissue disease (7).BPDCN can occur in individuals of all ages (range, 8 months-103 years), however, it predominantly occurs in the elderly (8). Skin involvement is the most prominent clinical feature and includes isolated, confined or generalized plaques or nodules. The plaque diameter ranges from a few millimeters to over ten centimeters, while the color ranges from dark red to characteristic purple, and ulcers occasionally occur. The manifestations of this disease also occasionally involve the mucosae (9,10). In addition to the initial manifestation of skin lesions, the disease involves other systems. For example, lymphadenectasis occurs in 40-50% of patients, the bone marrow and peripheral blood are involved in 60-90% of patient...