Abstract
INTRODUCTION: Acute myeloid leukemia (AML) with plasmacytoid dendritic cell differentiation (pDC-AML) is a newly described subtype of leukemia with features resembling blastic plasmacytoid dendritic cell neoplasm (BPDCN). CASE PRESENTATION: Herein, we present a case of pDC-AML in which bone marrow findings were best classified as AML, whereas cutaneous manifestations by morphology and immunophenotype were highly suggestive of a pDC neoplasm. RESULTS: A high-dose cytarabine with anthracycline backbone and venetoclax was administered based on efficacy in AML and BPDCN, respectively. The patient achieved complete remission as well as resolution of FDG-avid activity by PET-CT imaging. CONCLUSIONS: This report highlights that clinical correlation with immunophenotype and molecular testing is important in distinguishing these unique entities to guide appropriate diagnosis and management.