Blastic plasmacytoid dendritic cell neoplasm (BPDCN) — clinical features, diagnosis, and treatment in the experience from a single institution

Abstract

Introduction. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is rarely diagnosed proliferative disease with very poor prognosis originating from the precursors of plasmacytoid dendritic cells.

Materials and methods. In this study, we report on 7 patients diagnosed and treated at our institution between 2006 and 2012. Median age of patients was 68 years (range 37–78 years), and 4 were men. All patients presented with a skin lesion, with additional secondary lymph nodes (n = 3), peripheral blood (n = 1) and bone marrow involvement (n = 3). Disease was diagnosed based on typical immunophenotypical features in histopathological and flow cytometry examination of skin, blood or bone marrow (CD 4+/CD56+: n = 7, CD 123+: n = 3).

Results. Three patients were initially diagnosed with melanoma. Three of patients were treated with non-Hodgkin lymphoma-directed chemotherapy, three other — acute myeloid leukemia chemotherapy and one with acute lymphoblastic leukemia-type treatment. Median overall survival of non transplanted patients (n = 6) was 3.5 months (range 1.5–20 months), while patient allografted from related donor, treated with acute lymphoblastic leukemia-type chemotherapy, remains in complete remission until now (OS = 80 months).

Conclusion. This rare entity is a diagnostic challenge and needs dedicated clinical studies to establish appropriate therapy. Of note is a good outcome of a single patient after allogeneic stem cell transplantation.