International Journal of Clinical Science and Medical Research

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.

Blastic plasmacytoid dendritic cell neoplasm · BPDCN · Immunophenotyping · CD4 CD56 CD123 · Cytopenia

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