Blastic Plasmacytoid Dendritic Cell Neoplasm in an Adolescent Managed in a Resource-Limited Setting: A Case Report

Blastic plasmacytoid dendritic cell neoplasms (BPDCNs) are rare, aggressive haematologic malignancies, uncommon in adolescents and often difficult to diagnose. We report a 16-year-old male presenting with lymphadenopathy, pancytopenia and constitutional symptoms. Bone marrow aspirate noted 84% blasts with corresponding flow cytometry and lymph node biopsy supporting the diagnosis. Peripheral blood next-generation sequencing (NGS) revealed an NRAS p.(G12R) missense, gain-of-function mutation with a variant allele frequency (VAF) of 25%. The patient was treated with a combination of AML and ALL-type chemotherapy and succumbed to neutropenic sepsis 259 days after his diagnosis. This case highlights the challenges in diagnosis and treatment of BPDCN in a resource-limited setting.