Blastic plasmacytoid dendritic cell neoplasm: A rare subtype of myeloid leukemia

Abstract

A34-year-old male presented with a 2-month history of fever, backache, and generalized rash. Clinical examination revealed generalized lymphadenopathy along with maculopapular, erythematous, confluent rash involving the face, trunk, and extremities. Complete blood counts showed pancytopenia (hemoglobin 8.1 g/dL, platelet count 7000/mm, and white blood cell count 2000/mm). Bone marrow examination revealed intermediate to large sized blasts. The blast population expressed CD4, CD56, HLA-DR, dim to negative CD38 and CD45 and variable CD123. Blasts did not express CD 303, markers of myeloid, B-cell or T-cell lineage. The morphological and immunophenotypic findings were consistent with a diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN). BPDCN is a rare malignancy, and belongs to the category of acute myeloid leukemia and related precursor neoplasms. It commonly affects elderly males and involves skin, bone marrow and lymph nodes. The