Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): 2025 Update on Diagnosis, Pathophysiology, Risk Assessment, and Management.

OVERVIEW: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic neoplasm that involves multiple organs, including the skin, bone marrow and blood, lymph nodes, and central nervous system. Violaceous tumors or plaques appearing in sun-exposed areas are characteristic and present in most patients. The disease typically affects adult men aged 60-70 years, although children and younger adults may also be affected.

DIAGNOSIS: Diagnosis is based on biopsy of an involved site and is typically based on the identification of blastoid cells displaying the classical immunophenotypes CD123, CD4, and CD56 in addition to specific pDC markers.

PROGNOSIS: The median overall survival is 18-24 months. Potential risk factors for shortened survival include older age, overt bone marrow involvement, and genetic abnormalities.

THERAPY AND MANAGEMENT: Either chemotherapy-based regimens or tagraxofusp, a CD123-directed interleukin 3 conjugated with diphtheria toxin, may be used for upfront therapy. Eligible patients are recommended to be consolidated with hematopoietic allogeneic stem cell transplantation. Assessment and treatment of the central nervous system is mandatory, and careful monitoring of skin disease in conjunction with dermatology is essential.