Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant

Detalhes bibliográficos
Autor(a) principal: Alencar,Railene Célia B.
Data de Publicação: 2016
Outros Autores: Guimarães,Andréa M., Brito Junior,Lacy C.
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Jornal Brasileiro de Patologia e Medicina Laboratorial (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442016000500307
Resumo: ABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH, evolving in three years with Budd-Chiari syndrome and liver transplantation. Post-transplant complications, hepatic arterial thrombosis, graft rejection, liver retransplantation and treatment of PNH with eculizumab. Clinical stabilization and cessation of symptoms were achieved.
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spelling Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplantpure red-cell aplasiaparoxysmal hemoglobinurialiver transplantationABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH, evolving in three years with Budd-Chiari syndrome and liver transplantation. Post-transplant complications, hepatic arterial thrombosis, graft rejection, liver retransplantation and treatment of PNH with eculizumab. Clinical stabilization and cessation of symptoms were achieved.Sociedade Brasileira de Patologia Clínica2016-10-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442016000500307Jornal Brasileiro de Patologia e Medicina Laboratorial v.52 n.5 2016reponame:Jornal Brasileiro de Patologia e Medicina Laboratorial (Online)instname:Sociedade Brasileira de Patologia (SBP)instacron:SBP10.5935/1676-2444.20160051info:eu-repo/semantics/openAccessAlencar,Railene Célia B.Guimarães,Andréa M.Brito Junior,Lacy C.eng2016-12-06T00:00:00Zoai:scielo:S1676-24442016000500307Revistahttp://www.scielo.br/jbpmlhttps://old.scielo.br/oai/scielo-oai.php||jbpml@sbpc.org.br1678-47741676-2444opendoar:2016-12-06T00:00Jornal Brasileiro de Patologia e Medicina Laboratorial (Online) - Sociedade Brasileira de Patologia (SBP)false
dc.title.none.fl_str_mv Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
title Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
spellingShingle Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
Alencar,Railene Célia B.
pure red-cell aplasia
paroxysmal hemoglobinuria
liver transplantation
title_short Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
title_full Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
title_fullStr Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
title_full_unstemmed Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
title_sort Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
author Alencar,Railene Célia B.
author_facet Alencar,Railene Célia B.
Guimarães,Andréa M.
Brito Junior,Lacy C.
author_role author
author2 Guimarães,Andréa M.
Brito Junior,Lacy C.
author2_role author
author
dc.contributor.author.fl_str_mv Alencar,Railene Célia B.
Guimarães,Andréa M.
Brito Junior,Lacy C.
dc.subject.por.fl_str_mv pure red-cell aplasia
paroxysmal hemoglobinuria
liver transplantation
topic pure red-cell aplasia
paroxysmal hemoglobinuria
liver transplantation
description ABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH, evolving in three years with Budd-Chiari syndrome and liver transplantation. Post-transplant complications, hepatic arterial thrombosis, graft rejection, liver retransplantation and treatment of PNH with eculizumab. Clinical stabilization and cessation of symptoms were achieved.
publishDate 2016
dc.date.none.fl_str_mv 2016-10-01
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=S1676-24442016000500307
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442016000500307
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.5935/1676-2444.20160051
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
Sociedade Brasileira de Patologia Clínica
publisher.none.fl_str_mv
Sociedade Brasileira de Patologia Clínica
dc.source.none.fl_str_mv Jornal Brasileiro de Patologia e Medicina Laboratorial v.52 n.5 2016
reponame:Jornal Brasileiro de Patologia e Medicina Laboratorial (Online)
instname:Sociedade Brasileira de Patologia (SBP)
instacron:SBP
instname_str Sociedade Brasileira de Patologia (SBP)
instacron_str SBP
institution SBP
reponame_str Jornal Brasileiro de Patologia e Medicina Laboratorial (Online)
collection Jornal Brasileiro de Patologia e Medicina Laboratorial (Online)
repository.name.fl_str_mv Jornal Brasileiro de Patologia e Medicina Laboratorial (Online) - Sociedade Brasileira de Patologia (SBP)
repository.mail.fl_str_mv ||jbpml@sbpc.org.br
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