RESUMO
La histiocitosis de células de Langerhans (HCL) es una enfermedad caracterizada por la proliferación de células dendríticas CD1a+con compromiso orgánico único o difuso. La identificación de mutaciones génicas recurrentes ha confirmado la hipótesis de HCL como una verdadera neoplasia. La papulosis linfomatoide (PL) pertenece al espectro de los linfomas cutáneos primarios CD30+. La HCL ha sido descrita en asociación con otros trastornos linfoproliferativos, pero hasta la fecha, las lesiones constituidas por células de Langerhans (CL) han sido consideradas de carácter reactivo, relacionada con citocinas producidas por la interacción linfoma microambiente. Algunos autores designan estas lesiones «Langerhans cells like lesions». Presentamos el caso de una mujer de 28 años con HCL multisistémica y presencia simultánea de lesiones de PL con hiperplasia reactiva de CL
Langerhans cell histiocytosis (LCH) is a disease characterized by proliferation of CD1a+dendritic cells with local or diffuse organ compromise. The identification of recurrent gene mutations has confirmed the hypothesis of LCH as a true neoplasm. Lymphomatoid papulosis (LyP) belongs to the spectrum of CD30+primary cutaneous lymphomas. LCH has been described in association with other lymphoproliferative disorders. However, lesions constituted by Langerhans cells (LC) have been commonly considered reactive, related to cytokines produced by the lymphoma-microenvironment interaction. Some authors designate these lesions as "Langerhans cells-like lesions". We present the case of a 28-years-old woman with multisystem LCH and simultaneous PyL lesions with reactive LC hyperplasia
Assuntos
Humanos , Feminino , Adulto , Histiocitose de Células de Langerhans/patologia , Papulose Linfomatoide/patologia , Células Dendríticas/patologia , Transtornos Linfoproliferativos/patologia , Prednisona/uso terapêutico , Cladribina/uso terapêuticoRESUMO
Langerhans cell histiocytosis (LCH) is a disease characterized by proliferation of CD1a+dendritic cells with local or diffuse organ compromise. The identification of recurrent gene mutations has confirmed the hypothesis of LCH as a true neoplasm. Lymphomatoid papulosis (LyP) belongs to the spectrum of CD30+primary cutaneous lymphomas. LCH has been described in association with other lymphoproliferative disorders. However, lesions constituted by Langerhans cells (LC) have been commonly considered reactive, related to cytokines produced by the lymphoma-microenvironment interaction. Some authors designate these lesions as "Langerhans cells-like lesions". We present the case of a 28-years-old woman with multisystem LCH and simultaneous PyL lesions with reactive LC hyperplasia.
