Neoplasia blástica plasmocitoide de células dendríticas

La neoplasia blastica plasmocitoide de celulas dendriticas es un tumor poco frecuente que se presenta en adultos y tiene un curso clinico desfavorable. El cuadro inicial se manifiesta por afeccion cutanea y posteriormente evoluciona a una enfermedad extracutanea generalizada. Se presenta el caso de un varon con lesiones tumorales, algunas de ellas necroticas, con infiltrado monomorfo de celulas atipicas a nivel dedermis y con inmunohistoquimica CD3 negativo, y CD4, CD56 y CD123 positivos. El cuadro correspondio a una neoplasia blastica plasmocitoide de celulas dendriticas con buena respuesta inicial a quimioterapia, sin embargo a los 12 meses el paciente fallecio debido a un shock septico. Blastic plasmacytoid dendritic cell neoplasia is a rare tumor that occurs in adults with an unfavorable clinical course. Its initial presentation is characterized by skin condition which evolves later into a widespread extracutaneous disease. We report the case of a man with skin tumors, some of them necrotic, with atypical cells monomorphic infiltrate of the dermis and with an immunohistochemistry test negative to CD3, and positive to CD4, CD56 and CD123. The case was diagnosed as a blastic plasmacytoid dendritic cell neoplasia with a good initial response to chemotherapy, but the patient died 12 months later because of a septic shock.