Blastic plasmacytoid dendritic cell neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare invasive lymphoma, which was recognized as an independent entity in the 2008 World Health Organization (WHO) classification of cutaneous lymphomas. Cutaneous involvement often represents the first manifestation of this disease, which may appear as bruise-like nodules or plaques. It has a predilection for the invasion of bone marrow and lymph nodes with an aggressive course and poor prognosis. The tumor cells , which have been considered to derive from the precursors of plasmacytoid dendritic cells with special immunophenotype, are positive for CD4, CD56,plasmacytoid dendritic cell-associated antigens (CD123, TCL1 and BDCA-2), and partly positive to TdT and CD68. Currently, the aetiology of BPDCN remains unknown and there are no standard therapeutic schemes. Key words: Neoplasms; Dendritic cells; Plasma cells