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

Detalhes bibliográficos
Autor(a) principal: Vicari, Perla [UNIFESP]
Data de Publicação: 2006
Outros Autores: Figueiredo, Maria Stella [UNIFESP]
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Institucional da UNIFESP
Texto Completo: http://dx.doi.org/10.1590/S1516-84842006000100018
http://repositorio.unifesp.br/handle/11600/2974
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 intermediaRegressão da hematopoese extramedular na talassemia intermédia após terapia com hidroxiuréiaHemoglobinopathiesextramedullary hematopoiesisthalassemiahydroxyureaHemoglobinopatiahematopoese extramedulartalassemiahidroxiuréiaExcessive 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.A excessiva eritropoese ineficaz na talassemia pode causar hemato-poese extramedular (HEM), resultando em hepatomegalia, esplenomegalia e massas de tecido hematopoético em diversos tecidos. Localizações de HEM compensatória menos freqüentes são rins, glândulas adrenais, canal medular, pleura, pericárdio, duramáter, tecido adiposo e pele. Entretanto, HEM intratorácica é condição rara. Estratégias terapêuticas incluem radiação e transfusões sanguíneas. O uso de hidroxiuréia concomitante a terapêutica transfusional foi associado à regressão clínica da HEM na talassemia. Nós descrevemos um caso de HEM intratorácica em paciente portadora de talassemia intermédia, com significante regressão do quadro após terapêutica isolada com hidroxiuréia.Universidade Federal de São Paulo (UNIFESP)UNIFESPSciELOAssociação Brasileira de Hematologia e Hemoterapia e Terapia CelularUniversidade Federal de São Paulo (UNIFESP)Vicari, Perla [UNIFESP]Figueiredo, Maria Stella [UNIFESP]2015-06-14T13:32:01Z2015-06-14T13:32:01Z2006-03-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion71-72application/pdfhttp://dx.doi.org/10.1590/S1516-84842006000100018Revista Brasileira de Hematologia e Hemoterapia. Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular, v. 28, n. 1, p. 71-72, 2006.10.1590/S1516-84842006000100018S1516-84842006000100018.pdf1516-8484S1516-84842006000100018http://repositorio.unifesp.br/handle/11600/2974engRevista Brasileira de Hematologia e Hemoterapiainfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESP2024-08-04T15:19:44Zoai:repositorio.unifesp.br/:11600/2974Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestbiblioteca.csp@unifesp.bropendoar:34652024-08-04T15:19:44Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false
dc.title.none.fl_str_mv Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia
Regressão da hematopoese extramedular na talassemia intermédia após terapia com hidroxiuréia
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 [UNIFESP]
Hemoglobinopathies
extramedullary hematopoiesis
thalassemia
hydroxyurea
Hemoglobinopatia
hematopoese extramedular
talassemia
hidroxiuréia
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 [UNIFESP]
author_facet Vicari, Perla [UNIFESP]
Figueiredo, Maria Stella [UNIFESP]
author_role author
author2 Figueiredo, Maria Stella [UNIFESP]
author2_role author
dc.contributor.none.fl_str_mv Universidade Federal de São Paulo (UNIFESP)
dc.contributor.author.fl_str_mv Vicari, Perla [UNIFESP]
Figueiredo, Maria Stella [UNIFESP]
dc.subject.por.fl_str_mv Hemoglobinopathies
extramedullary hematopoiesis
thalassemia
hydroxyurea
Hemoglobinopatia
hematopoese extramedular
talassemia
hidroxiuréia
topic Hemoglobinopathies
extramedullary hematopoiesis
thalassemia
hydroxyurea
Hemoglobinopatia
hematopoese extramedular
talassemia
hidroxiuréia
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
2015-06-14T13:32:01Z
2015-06-14T13:32:01Z
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://dx.doi.org/10.1590/S1516-84842006000100018
Revista Brasileira de Hematologia e Hemoterapia. Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular, v. 28, n. 1, p. 71-72, 2006.
10.1590/S1516-84842006000100018
S1516-84842006000100018.pdf
1516-8484
S1516-84842006000100018
http://repositorio.unifesp.br/handle/11600/2974
url http://dx.doi.org/10.1590/S1516-84842006000100018
http://repositorio.unifesp.br/handle/11600/2974
identifier_str_mv Revista Brasileira de Hematologia e Hemoterapia. Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular, v. 28, n. 1, p. 71-72, 2006.
10.1590/S1516-84842006000100018
S1516-84842006000100018.pdf
1516-8484
S1516-84842006000100018
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Revista Brasileira de Hematologia e Hemoterapia
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 71-72
application/pdf
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 reponame:Repositório Institucional da UNIFESP
instname:Universidade Federal de São Paulo (UNIFESP)
instacron:UNIFESP
instname_str Universidade Federal de São Paulo (UNIFESP)
instacron_str UNIFESP
institution UNIFESP
reponame_str Repositório Institucional da UNIFESP
collection Repositório Institucional da UNIFESP
repository.name.fl_str_mv Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)
repository.mail.fl_str_mv biblioteca.csp@unifesp.br
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