Abstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy. The neoplasm is associated with a poor prognosis and frequent diagnostic challenges, mainly due to its cutaneous manifestations that can delay an accurate diagnosis or lead to its misclassification as other hematological disorders. In the absence of standardized protocols, the management of BPDCN relies on crucial judgment based on individual cases. The present study reports the case of a 51-year-old male with BPDCN who presented with a fever and bilateral limb pain, and achieved complete remission after only one cycle of azacitidine-venetoclax (VA) therapy. As the patient required concurrent administration of posaconazole (a potent CYP3A4 inhibitor), the dose of venetoclax was adjusted to a low dose of 200 mg per day, and the patient still achieved successful disease remission. The findings from this case study highlight the potential of VA therapy in the management of BPDCN. The study also emphasizes the diversity of the clinical manifestations of BPDCN and the crucial role of combination therapy in its management.