title: Primary cutaneous blastic plasmacytoid dendritic cell neoplasm without extracutaneous manifestation: case report and review of the literature.
abstract: Blastic plasmacytoid dendritic cell (BPDC) neoplasm is a rare, highly aggressive hematopoietic malignancy with involvement of bone marrow and peripheral blood. We present 2 cases of primary cutaneous BPDC neoplasm without extracutaneous manifestation during the course of disease. A 36-year-old and a 51-year-old male presented with erythematous patches, purple plaques, and nodules on the head, trunk, and extremities. Skin biopsies revealed that the lesions of both cases were composed of diffusely medium-sized monomorphic blastoid cells infiltrating into the dermis and subcutis. The neoplastic cells were strongly positive for CD4, CD56, CD123, and TdT, whereas other T-cell markers and EBV markers were not expressed. The patients underwent polychemotherapy with hyper-CVAD regimen and obtained a remarkable clinical response with regression of skin lesions. No sign of recurrence and extracutaneous manifestation was found during the period of follow-up. We presume that the favorable prognosis of our cases might result from the presentation only with a skin lesion, diffuse TdT expression in tumor cells, and aggressive chemotherapy with hyper-CVAD regimens. Laboratory examination for blood and bone marrow should be performed every 3-6 months during the first period of follow-up to monitor the progression of disease even if the patients had complete remission at initial chemotherapy.
authors.affiliation: Department of Pathology, the First Affiliated Hospital, Sun Yat-sen University, 58# Zhongshan Road II, Guangzhou, Guangdong 510080, China.
authors.forename: Yang
authors.initials: Y
authors.lastname: Li
authors.forename: Zhi
authors.initials: Z
authors.lastname: Li
authors.forename: Han-liang
authors.initials: HL
authors.lastname: Lin
authors.forename: Xiao-hong
authors.initials: XH
authors.lastname: Chen
authors.forename: Bin
authors.initials: B
authors.lastname: Li
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D015704:CD4 Antigens / Q000276:immunology; D019002:CD56 Antigen / Q000032:analysis / Q000276:immunology; D003520:Cyclophosphamide / Q000627:therapeutic use; D003713:Dendritic Cells / Q000276:immunology / Q000473:pathology*; D003907:Dexamethasone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004317:Doxorubicin / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D012074:Remission Induction; D012867:Skin / Q000276:immunology / Q000473:pathology; D012878:Skin Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D015704:CD4 Antigens; D019002:CD56 Antigen; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide; D008727:Methotrexate