Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia

Detalhes bibliográficos
Autor(a) principal: Vicari,Perla
Data de Publicação: 2006
Outros Autores: Figueiredo,Maria Stella
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Revista brasileira de hematologia e hemoterapia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842006000100018
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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spelling Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermediaHemoglobinopathiesextramedullary hematopoiesisthalassemiahydroxyureaExcessive 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.Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular2006-03-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842006000100018Revista Brasileira de Hematologia e Hemoterapia v.28 n.1 2006reponame:Revista brasileira de hematologia e hemoterapia (Online)instname:Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)instacron:ABHHTC10.1590/S1516-84842006000100018info:eu-repo/semantics/openAccessVicari,PerlaFigueiredo,Maria Stellaeng2006-08-10T00:00:00Zoai:scielo:S1516-84842006000100018Revistahttp://www.rbhh.org/pt/archivo/https://old.scielo.br/oai/scielo-oai.phpsbhh@terra.com.br||secretaria@rbhh.org1806-08701516-8484opendoar:2006-08-10T00:00Revista brasileira de hematologia e hemoterapia (Online) - Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)false
dc.title.none.fl_str_mv Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
title Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
spellingShingle Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
Vicari,Perla
Hemoglobinopathies
extramedullary hematopoiesis
thalassemia
hydroxyurea
title_short Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
title_full Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
title_fullStr Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
title_full_unstemmed Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
title_sort Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
author Vicari,Perla
author_facet Vicari,Perla
Figueiredo,Maria Stella
author_role author
author2 Figueiredo,Maria Stella
author2_role author
dc.contributor.author.fl_str_mv Vicari,Perla
Figueiredo,Maria Stella
dc.subject.por.fl_str_mv Hemoglobinopathies
extramedullary hematopoiesis
thalassemia
hydroxyurea
topic Hemoglobinopathies
extramedullary hematopoiesis
thalassemia
hydroxyurea
description 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.
publishDate 2006
dc.date.none.fl_str_mv 2006-03-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format report
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842006000100018
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842006000100018
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1590/S1516-84842006000100018
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular
publisher.none.fl_str_mv Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular
dc.source.none.fl_str_mv Revista Brasileira de Hematologia e Hemoterapia v.28 n.1 2006
reponame:Revista brasileira de hematologia e hemoterapia (Online)
instname:Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
instacron:ABHHTC
instname_str Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
instacron_str ABHHTC
institution ABHHTC
reponame_str Revista brasileira de hematologia e hemoterapia (Online)
collection Revista brasileira de hematologia e hemoterapia (Online)
repository.name.fl_str_mv Revista brasileira de hematologia e hemoterapia (Online) - Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
repository.mail.fl_str_mv sbhh@terra.com.br||secretaria@rbhh.org
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