Résumé
Intracraneal manifestations of Hodgkin's Disease (HD) are extremely rare, with an estimated incidence rate of approximately 0.5%. They can be classified as: 1) treatment-related leucoencephalopathy, 2) central nervous system infections, 3) paraneoplasic syndromes and 4) intracraneal lymphomas, which could be sub-classified into intraparenchymal or intradural masses. We describe a case of a 40 year-old male with mixed cellularity type HD who developed neurological manifestations as relapsed disease. Magnetic resonance imaging suggested leptomeningeal metastases and atypical cells were found in cerebrospinal fluid. The patient died from progressive disease refractory to third line chemotherapy. There are less than 50 similar cases reported in the literature. We review the clinical features and differential diagnosis of leptomeningeal metastases in Hodgkin's disease.
Sujets)
Adulte , Humains , Mâle , Maladie de Hodgkin/anatomopathologie , Tumeurs des méninges/secondaire , Ponction-biopsie à l'aiguille , Bléomycine/administration et posologie , Cyclophosphamide/administration et posologie , Cisplatine/administration et posologie , Cytarabine/administration et posologie , Diagnostic différentiel , Dacarbazine/administration et posologie , Maladie de Hodgkin/liquide cérébrospinal , Maladie de Hodgkin/traitement médicamenteux , Doxorubicine/administration et posologie , Étoposide/administration et posologie , Issue fatale , Leucoencéphalopathie multifocale progressive/induit chimiquement , Leucoencéphalopathie multifocale progressive/anatomopathologie , Imagerie par résonance magnétique , Tumeurs des méninges/liquide cérébrospinal , Prednisone/administration et posologie , Procarbazine/administration et posologie , Protocoles de polychimiothérapie antinéoplasique/administration et posologie , Protocoles de polychimiothérapie antinéoplasique/usage thérapeutique , Récidive , Syndromes paranéoplasiques/anatomopathologie , Vinblastine/administration et posologie , Vincristine/administration et posologieRésumé
La amiloidosis se caracteriza por el depósito tisular, localizado o sistémico, de una sustancia formada por tres fracciones: 1) proteica, 2) mucopolisacáridos, 3) lípidos e hierro, con ultraesctructura fibrilar. Son fibrillas finas, rígidas, con conformación cristalográfica beta, que le da sus propriedades especificas. Se individualizan cuatro tipos de amiloide derivados de la cadenas lovianas y otras inmunoglobulinas, aminoácidos o sustancias similares a la prealbúmina. Si todos los depósitos con acúmulos de fibrilas proteicas, una de sus causas sería la síntesisexcesiva de ellas. Esto no es suficiente y se requiere que estas proteínas precursosras sean captadas por los macrófagos, sometidas a proteolisis y por exocitosis depositadas extracelularmente. Se presenta una paciente de 55 años, en quien la participación hepática y renal hace sospechar uns amiloidosis que se confirma por hallarse una gamapatía monoclonal y por biopsia de tejidos
Sujets)
Adulte d'âge moyen , Humains , Femelle , Amyloïdose/diagnosticRésumé
Amyloidosis is distinguished by store both localized or systemic, of a substance conformed for 3 fraction: 1) protein (90
), 2) Mucopolysaccharides, 3) fatty and iron, with filament ultrastructure. These filaments are thin and rigid, with beta crystallographic shape. Four pattern of amyloid are distinguished. They are derived of light chains or another immunoglobulins, aminoacids or prealbumin like substance. If all deposits are protein filaments storage, one of their trials would be increased synthesis of them. This is not sufficient and need that these precursor proteins be attrached by macrophages they are subjected to proteolysis and are enclosed extracellularly. We present a female, 55 years old. The hepatic and renal participation permit to suspect an amyloidosis who is confirmed by the presence of monoclonal gammopathy and by biopsy of tissues.