title: Blastic plasmacytoid dendritic cell neoplasm.
abstract: Blastic plasmacytoid dendritic cell neoplasm is a rare and aggressive hematodermic neoplasia with frequent cutaneous involvement and leukemic dissemination. We report the case of a 76-year-old man with a 2 month history of violaceous nodules and a tumor with stony consistency, located on the head, and mandibular, cervical and supraclavicular lymphadenopathies. Multiple thoracic and abdominal adenopathies were identified on computerized tomography. Flow cytometry analysis of the skin, lymph node and bone marrow biopsies demonstrated the presence of plasmocytoid dendritic cell neoplastic precursor cells (CD4+, CD45+, CD56+ and CD123+ phenotype). After initial clinical and laboratorial complete remission with chemotherapy, the patient died due to relapse of the disease associated with the appearance of a cervical mass with medullary compromise.
authors.affiliation: Centro Hospitalar de Lisboa Central, EPE, Hospital de Santo António dos Capuchos, Dermatology Service, Lisbon, Portugal.
authors.forename: André
authors.initials: A
authors.lastname: Lencastre
authors.affiliation: Hospital Center of Central Lisbon, EPE, Santo António dos Capuchos Hospital, Dermatology Service, Lisbon, Portugal.
authors.forename: Joana
authors.initials: J
authors.lastname: Cabete
authors.affiliation: Centro Hospitalar de Lisboa Central, EPE, Hospital de Santo António dos Capuchos, Dermatology Service, Lisbon, Portugal.
authors.forename: Alexandre
authors.initials: A
authors.lastname: João
authors.affiliation: Centro Hospitalar de Lisboa Central, EPE, Hospital de Santo António dos Capuchos, Pathology Department, Service of Pathological Anatomy, Lisbon, Portugal.
authors.forename: Pedro
authors.initials: P
authors.lastname: Farinha
authors.affiliation: Centro Hospitalar de Lisboa Central, EPE, Hospital de Santo António dos Capuchos, Lisbon, Portugal.
authors.forename: Gilda
authors.initials: G
authors.lastname: Ferreira
authors.affiliation: Centro Hospitalar de Lisboa Central, EPE, Hospital de Santo António dos Capuchos, Dermatology Service, Lisbon, Portugal.
authors.forename: Sara
authors.initials: S
authors.lastname: Lestre
mesh_terms: D000368:Aged; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003713:Dendritic Cells / Q000473:pathology*; D017809:Fatal Outcome; D005434:Flow Cytometry; D006801:Humans; D007938:Leukemia / Q000473:pathology*; D008297:Male; D009361:Neoplasm Invasiveness; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology*