RESUMO
La enfermedad de Erdheim-Chester es una histiocitosis sistémica de origen desconocido, histológicamente distinta de la histiocitosis de células de Langerhans, que se caracteriza por lesiones óseas esclerosantes de distribución simétrica, con predominio en las diáfisis y metáfisis de huesos largos. No está clasificada dentro de las histiocitosis malignas, no obstante, su curso clínico suele ser agresivo, con escasa respuesta a los diversos tratamientos ensayados. Excepcionalmente, se han descrito casos con hallazgos anatomopatológicos de la enfermedad de ErdheimChester asociados a la presencia de histiocitos de Langerhans. Presentamos un nuevo paciente en el que se identificaron cambios radiográficos compatibles con enfermedad de Erdheim-Chester pero cuyo estudio histológico mostró una histiocitosis de células de Langerhans y cursó con ostealgias, mialgias, diabetes insípida, exoftalmos, lesiones óseas esclerosantes bilaterales y simétricas así como un síndrome cerebeloso (AU)
Assuntos
Masculino , Humanos , Pessoa de Meia-Idade , Histiocitose de Células de Langerhans , Doença de Erdheim-ChesterRESUMO
Erdheim-Chester disease is an idiopathic systemic histiocytosis hystologically different to Langerhans histiocytosis which is characterized by symmetric sclerotic bone lesions, predominantly affecting the diaphyseal and metaphyseal areas of large bones. It is not classified within malignant histiocytosis, but the clinical course tends to be aggressive with a poor response to different schedules of treatment. In rare cases the association of both entities has been reported: Erdheim-Chester disease and Langerhans histiocytosis. We present a new case with histological data of both histiocytosis whose clinical course included bone and muscle pain, insipidus diabetes, exophthalmos, bilateral symmetrical sclerosing bone lesions and a cerebellar syndrome.
Assuntos
Doença de Erdheim-Chester/complicações , Histiocitose de Células de Langerhans/complicações , Doença de Erdheim-Chester/diagnóstico , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
La compresión mielorradicular es infrecuente en el curso de los linfomas no hodgkinianos, generalmente ocurre por afección extradural y rara vez constituye su manifestación inicial. Aunque puede implicarse cualquier segmento espinal, se ha descrito cierto predominio en la región dorsal. En la mayoría de los casos se trata de linfomas de inmunofenotipo B de alto grado y su comportamiento es agresivo. En el presente trabajo aportamos los casos de seis adultos, cinco mujeres y un varón, en quienes las complicaciones neurológicas espinales constituyeron la primera manifestación de un linfoma no hodgkiniano y procedemos a revisar la literatura sobre estos procesos. (AU)
Assuntos
Pessoa de Meia-Idade , Adulto , Idoso , Masculino , Feminino , Humanos , Compressão da Medula Espinal , Linfoma não Hodgkin , Neoplasias da Medula EspinalRESUMO
Spinal cord compression and radicular involvement are infrequent events in the natural history of non-Hodgkin's lymphoma, which usually are secondary to invasion of spinal extradural space, and rarely are the presenting manifestations of this condition. Thoracic segment is predominantly affected, but any spinal region can be affected. Histologically, the spinal tumour are frequently a high-grade B lymphoma with aggressive behaviour. The aim of this work was to describe six new cases of non-Hodgkin's lymphoma in adults (five woman and one men), presenting with spinal neurological complications. The medical literature about this entity are revised.
Assuntos
Linfoma não Hodgkin/complicações , Compressão da Medula Espinal/etiologia , Neoplasias da Medula Espinal/complicações , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
The angiofollicular lymph node hyperplasia (Castleman's disease) is an entity of unknown etiology. Its diagnosis is based on clinical and histological criteria. We present the case of a 20-year-old male patient with asymmetric oligoarthritis and fever which response to nonsteroidal antiinflammatory drugs as onset of abdominal Castleman's disease mixed type.
