Résumé
Context: Spinal cord compression due to extramedullary hematopoiesis is a well-described but rare syndrome encountered in several clinical hematologic disorders, including beta-thalassemia. Case Report: We report the case of a patient with intermediate beta-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. Discussion: Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms.
Sujets)
Humains , Femelle , Adulte , Syndrome de compression médullaire/radiothérapie , Hématopoïèse extramédullaire/effets des radiations , bêta-Thalassémie/complications , Études de suiviRésumé
Relatamos o caso de um paciente, de 28 anos, portador de nevo melanocítico gigante congênito(NMGC), com transformaçao em melanoma e metátase no sistema nervoso central (SNC). Descreveremos resumidamente as características patológicas de ambas as lesoes (NMGC e melanoma), a frequência de malignizaçao no NMGC, os órgaos mais frequentemente acometidos pelas metástases do melanoma - dando ênfase ao acometimento do SNC - além dos fatores que podem levar à malignizaçao do NMGC, os exames usados para o diagnóstico das metástases no SNC - ressaltando a importância do líquido cefalorraquiano - e algumas modalidades terapêutica para o melanoma com acometimento do SNC.