Abstract
Blastic plasmacytoid dendritic cell neoplasm is a rare hematologic neoplasm characterized by cutaneous, hematologic, and central nervous system (CNS) involvement with poor prognosis. Diagnosis is made by flow cytometry, although there are no specific markers, making its diagnosis challenging. So far, with the available evidence, acute lymphoid leukemia-type schemes and consolidation with allogeneic transplant seem to become the first-line therapy. With its characterization, new therapies directed toward CD123 and the anti-apoptotic protein Bcl-2 have appeared to prolong the survival of these patients. We present a case of a 27-year-old male patient diagnosed with blastic plasmacytoid dendritic cell neoplasm with unusual CNS manifestations and without skin involvement who achieved complete remission with venetoclax and improvement of neurological symptoms, making him a candidate for hematopoietic stem cell transplant.