Blastic plasmacytoid dendritic cell neoplasm: a single-center experience.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematologic malignancy originating from the precursor of the plasmacytoid dendritic cell. It is very rare and has only recently been recognized as a distinct entity. In this study, we report our experience of BPDCN to review the clinical, pathological features and treatment outcomes. A database at the Asan Medical Center was screened for all patients with BPDCN treated between 2000 and 2010. Seven patients were confirmed as BPDCN and included in this analysis. The median age of the patients was 40 years (range, 18-62 years), and four patients were male. Sites of disease involvement included the skin (n = 4), lymph node (n = 4), and peripheral blood/bone marrow (n = 2). Tumor cells were positive for CD4 (n = 5), CD56 (n = 6), and CD123 (n = 7). Six patients received multi-agent chemotherapy as first-line treatment, while one patient was given radiotherapy. The median progression-free survival was 8.6 months (range, 2.6-28.9 months) and overall survival was 15.1 months (range, 4.4-60.0 months) with a median follow-up period of 13.8 months (range, 1.9-29.9 months). Notably, all four patients with cutaneous involvement survived, whereas those without skin involvement succumbed to death, even though two of them were given salvage chemotherapy. In this study, patients with BPDCN showed various clinical, histological, and immunophenotypical features. Our experience warrants further examination of the prognostic significance of skin involvement in BPDCN.