Neoplasia de células dendríticas plasmocitoides blásticas con respuesta inicial a quimioterapia y recaída en sistema nervioso central: Caso clínico

• Loreto Contreras ^[2] ; Loreto Mercado ^[3] ; Carolina Delgado ^[2] ; Claudia Cabezas ^[2] ; Laksmi Starke ^[2] ; Mónica Romero ^[3] ; Fernando Ibieta ^[3] ; Mauricio Henríquez ^[1] ; Mauricio Chandia ^[2]
  1. [1] Universidad de Concepción

     Universidad de Concepción

     Comuna de Concepción, Chile

     
  2. [2] Hospital Dr. Guillermo Grant Benavente Unidad de Anatomía Patológica
  3. [3] Hospital Dr. Guillermo Grant Benavente Unidad de Hematología

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• Localización: Revista Médica de Chile, ISSN-e 0034-9887, Vol. 145, Nº. 1, 2017, págs. 115-120
• Idioma: español
• Títulos paralelos:
  • Blastic plasmacytoid dendritic cell neoplasm with complete clinical remission with chemotherapy and central nervous system relapse: Report of one case
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• Resumen

  • 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.