When the skin speaks first, blastic plasmacytoid dendritic cell neoplasm: A rare and therapeutically challenging hematological malignancy

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an infrequently encountered hematological malignancy derived from precursor plasmacytoid dendritic cells. In clinical practice, it is most often recognized because of its striking cutaneous manifestations and its tendency to relapse despite initial treatment responses. Long-term survival is there only after allogeneic hematopoietic stem cell transplantation (allo-HSCT) can be offered. We report a 61-year-old male with relapsed BPDCN presenting predominantly with skin involvement. The patient achieved complete remission following salvage chemotherapy with a HyperCVAD regimen and subsequently underwent Haplo-HSCT. This case shown the diagnostic complexity of BPDCN, its frequent treatment resistance, and the importance of timely transplantation in relapsed disease.