Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
Excessive ineffective erythropoiesis in thalassemia intermedia may cause extramedullary hematopoiesis (EMH), resulting in spleen and liver enlargement or masses in several tissues, mainly paravertebrally. Other less frequent locations of diffuse compensatory EMH are kidneys, adrenal glands, breasts,...
| Autores: | , |
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| Formato: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2006 |
| País: | Brasil |
| Recursos: | Universidade Federal de São Paulo (UNIFESP) |
| Repositorio: | Repositório Institucional da UNIFESP |
| Idioma: | inglés |
| OAI Identifier: | oai:repositorio.unifesp.br:11600/2974 |
| Acesso em linha: | http://dx.doi.org/10.1590/S1516-84842006000100018 http://repositorio.unifesp.br/handle/11600/2974 |
| Access Level: | acceso abierto |
| Palavra-chave: | Hemoglobinopathies extramedullary hematopoiesis thalassemia hydroxyurea Hemoglobinopatia hematopoese extramedular talassemia hidroxiuréia |
| Resumo: | Excessive ineffective erythropoiesis in thalassemia intermedia may cause extramedullary hematopoiesis (EMH), resulting in spleen and liver enlargement or masses in several tissues, mainly paravertebrally. Other less frequent locations of diffuse compensatory EMH are kidneys, adrenal glands, breasts, spinal cord, pleura, pericardium, duramater, adipose tissue and skin, although intrathoracic extramedullary hematopoiesis is a rare condition. Management strategies have included radiation and transfusion therapy. Hydroxyurea with transfusion therapy has been associated with clinical regression of EMH in thalassemia. We report an uncommon case of intrathoracic EMH in a patient with beta-thalassemia intermedia, that showed significant recovery with HU therapy. |
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