P272 : A woman with blastic plasmacytoid dendritic cell neoplasm on the face

A 47-year-old woman presented with 6-month history of a cutaneous lesion located on the right cheek. Physical examination revealed a well-demarcated violaceous multiple nodules with hardened brownish patch. She was otherwise healthy and presented no systemic symptoms. Histopathologic findings showed that numerous tumor cells were distributed in the dermis. The tumor cells were monomorphic medium sized atypical lymphocytes containing irregular round or indented nuclei with pale cytoplasm. Immunohistochemical stains were positive for CD4, CD56, CD68, Ki-67, terminal deoxynucleotidyl transferase, Bcl-2, leukocyte common antigen and negative for CD3, CD20, CD10, myeloperoxidase. Based on clinical and histopathologic findings, the cutaneous lesion was finally diagnosed as blastic plasmacytoid dendritic cell neoplasm (BPDCN). The patient’s peripheral blood chemistry values were within normal ranges and peripheral blood smear showed no abnormality. But bone marrow aspiration cytology and section biopsy showed involvement of malignant tumor cells. She admitted to hemato-oncology and received induction chemotherapy with modified hyper-CVAD (cyclophosphamide, vincristine, Adriamycin, dexamethasone). After consolidation treatment, she underwent allogenic stem cell transplantation a month ago. Here in we report a rare case of BPCDN because it is noteworthy that an isolated mild skin lesion was the initial symptom of this potentially lethal disease