Plasmacytoid Dendritic Cell-Derived Leukemia: A Case Report

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy (<1% of acute leukemias), predominantly affecting elderly patients. It typically presents with cutaneous lesions associated with bone marrow and peripheral blood involvement. We report an atypical pediatric case in an 11-month-old infant without skin lesions. The complete blood count revealed cytopenias with leukocytosis, and bone marrow examination showed 89% blasts. Immunophenotyping demonstrated a CD4+, CD56+, CD123+, HLA-DR+ profile, consistent with BPDCN. Diagnosis relies primarily on immunophenotyping due to the absence of exclusive specific markers. Management is not standardized, but ALL-type regimens and allogeneic stem cell transplantation may improve prognosis. This case highlights the diagnostic challenges in pediatric presentations and the importance of a multidisciplinary approach.