title: [Blastic plasmacytoid dendritic cell neoplasm with complete clinical remission with chemotherapy and central nervous system relapse: Report of one case].
abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. The demonstration of tumor cells with the characteristic immunophenotype with expression of CD56, generally CD4 and dendritic cell antigens (CD123, cyTCL-1, HLA-DR), in the absence of myeloid or lymphoid lineage markers is required for the diagnosis. Responses to chemotherapy are initially satisfactory, with frequent systemic and central nervous system relapses. We report a 24 year-old male with BPDCN, initially diagnosed and treated as non-Hodgkin CD4+ T-cell lymphoma, with initial complete remission who evolved with early central nervous system relapse. A second attempt of chemotherapy failed and the patient died two months later.
authors.affiliation: Unidad de Anatomía Patológica, Hospital Dr. Guillermo Grant Benavente, Concepción, Chile.
authors.forename: Loreto
authors.initials: L
authors.lastname: Contreras
authors.affiliation: Unidad de Hematología, Hospital Dr. Guillermo Grant Benavente, Concepción, Chile.
authors.forename: Loreto
authors.initials: L
authors.lastname: Mercado
authors.affiliation: Unidad de Anatomía Patológica, Hospital Dr. Guillermo Grant Benavente, Concepción, Chile.
authors.forename: Carolina
authors.initials: C
authors.lastname: Delgado
authors.affiliation: Unidad de Anatomía Patológica, Hospital Dr. Guillermo Grant Benavente, Concepción, Chile.
authors.forename: Claudia
authors.initials: C
authors.lastname: Cabezas
authors.affiliation: Unidad de Anatomía Patológica, Hospital Dr. Guillermo Grant Benavente, Concepción, Chile.
authors.forename: Laksmi
authors.initials: L
authors.lastname: Starke
authors.affiliation: Unidad de Hematología, Hospital Dr. Guillermo Grant Benavente, Concepción, Chile.
authors.forename: Mónica
authors.initials: M
authors.lastname: Romero
authors.affiliation: Unidad de Hematología, Hospital Dr. Guillermo Grant Benavente, Concepción, Chile.
authors.forename: Fernando
authors.initials: F
authors.lastname: Ibieta
authors.affiliation: Facultad de Medicina, , Universidad de Concepción, Concepción, Chile.
authors.forename: Mauricio
authors.initials: M
authors.lastname: Henríquez
authors.affiliation: Unidad de Anatomía Patológica, Hospital Dr. Guillermo Grant Benavente, Concepción, Chile.
authors.forename: Mauricio
authors.initials: M
authors.lastname: Chandia
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016543:Central Nervous System Neoplasms / Q000556:secondary*; D003713:Dendritic Cells / Q000473:pathology*; D018450:Disease Progression; D017809:Fatal Outcome; D019337:Hematologic Neoplasms / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D012074:Remission Induction; D055815:Young Adult