Portrait of a rare blood cancer: blastic plasmacytoid dendritic cell neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematological malignancy derived from immature plasmacytoid dendritic cells. This case report discusses a 72-year-old male patient who presented with a “raised blood bruise” on his back, later diagnosed as BPDCN. Initial symptoms included a violaceous, indurated tumor and additional plaques without systemic symptoms. His medical history was notable for hemochromatosis and a family history of pancreatic and lung cancer. A 4-mm punch biopsy confirmed BPDCN. The patient initially sought treatment at a cancer center but chose to receive standard care closer to his home, undergoing 5 cycles of tagraxofusp with complications including pleural effusion and neutropenia. A positron emission tomography (PET)/computed tomography (CT) scan in December 2023 indicated clinical remission, with BPDCN confined to the skin. In February 2024, the patient received high-dose chemotherapy and an allogeneic stem cell transplant at MD Anderson. Post-transplant complications included chemotherapy-related toxicities, aspiration pneumonia, atrial fibrillation, and hypervolemia. Despite intensive care, the patient developed severe complications, and resuscitation efforts were ultimately ceased in accordance with his prior wishes. This case highlights the complexity of BPDCN management, the potential for severe treatment-related complications, and the importance of individualized patient care plans.