Blast and Bursts: Unveiling Splenic Rupture in Blastic Plasmacytoid Dendritic Cell Neoplasia.

Abstract

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic cancer, accounting for less than 1% of acute leukemias in the U.S. Diagnosis involves detecting markers like CD123, CD4, CD56, TCL1, and TCF4. Treatment typically involved acute leukemia therapies, but Tagraxofusp, a targeted therapy, was recently approved. Despite advancements, prognosis remains grim, with a median survival of around 1 year. Atraumatic splenic rupture (ASR) is a rare complication of this condition, with only five cases reported from 1994 to 2018. Here we present a case of BPDCN complicated by ASR. This case emphasizes the challenges of diagnosing and treating BPDCN, noting its rarity and absence of standard therapy. Tagraxofusp has shown promising results but presents safety concerns like capillary leak syndrome, particularly in elderly patients with comorbidities.