Evans syndrome in male lupus patient: case report

ABSTRACT Evans syndrome (ES) is a rare autoimmune disease of unknown etiology. ES occurs when there is a combination of autoimmune hemolytic anemia (AIHA) with thrombocytopenia, whether or not accompanied by immune neutropenia. This syndrome fits into a special variable type of warm AHAI and may be...

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Autores: Dalmaso,Barbara F., Cortez,Afonso José P., Velasquez,Maitê Alexandra E., Silva Neto,Warlindo C., Silva,Flávio Aparecido R., Sousa,Lívia Mara S.
Tipo de recurso: informe técnico
Estado:Versión publicada
Fecha de publicación:2019
País:Brasil
Institución:Sociedade Brasileira de Patologia (SBP)
Repositorio:Jornal Brasileiro de Patologia e Medicina Laboratorial (Online)
Idioma:inglés
OAI Identifier:oai:scielo:S1676-24442019000400416
Acceso en línea:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442019000400416
Access Level:acceso abierto
Palabra clave:systemic lupus erythematosus
hemolytic autoimmune anemia
thrombocytopenia
lúpus eritematoso sistêmico
anemia hemolítica autoimune
trombocitopenia
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spelling Evans syndrome in male lupus patient: case reportsystemic lupus erythematosushemolytic autoimmune anemiathrombocytopenialúpus eritematoso sistêmicoanemia hemolítica autoimunetrombocitopeniaABSTRACT Evans syndrome (ES) is a rare autoimmune disease of unknown etiology. ES occurs when there is a combination of autoimmune hemolytic anemia (AIHA) with thrombocytopenia, whether or not accompanied by immune neutropenia. This syndrome fits into a special variable type of warm AHAI and may be related to rheumatic diseases, such as systemic lupus erythematosus (SLE). Its pathogenesis is still far away to be fully understood. Direct Coombs test results are positive in 98% of cases. ES treatment is required when the anemia due to hemolysis is intense, and corticotherapy is recommended initially. In refractory cases, splenectomy or rituximab therapy, as well as other immunosuppressants, such as azathioprine, may be used. In this study, we present the case of a 45-year-old patient, male, who was diagnosed with ES associated with active SLE. The patient was admitted to the emergency room presenting severe hemolytic anemia and onset of thrombocytopenia. He was treated with intravenous corticosteroid therapy, followed by azathioprine.Sociedade Brasileira de Patologia Clínica2019-08-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442019000400416Jornal Brasileiro de Patologia e Medicina Laboratorial v.55 n.4 2019reponame:Jornal Brasileiro de Patologia e Medicina Laboratorial (Online)instname:Sociedade Brasileira de Patologia (SBP)instacron:SBP10.5935/1676-2444.20190038info:eu-repo/semantics/openAccessengDalmaso,Barbara F.Cortez,Afonso José P.Velasquez,Maitê Alexandra E.Silva Neto,Warlindo C.Silva,Flávio Aparecido R.Sousa,Lívia Mara S.2019-08-28T00:00:00Zoai:scielo:S1676-24442019000400416Revistahttp://www.scielo.br/jbpmlhttps://old.scielo.br/oai/scielo-oai.php||jbpml@sbpc.org.br1678-47741676-2444opendoar:2019-08-28T00:00Jornal Brasileiro de Patologia e Medicina Laboratorial (Online) - Sociedade Brasileira de Patologia (SBP)false
dc.title.none.fl_str_mv Evans syndrome in male lupus patient: case report
title Evans syndrome in male lupus patient: case report
spellingShingle Evans syndrome in male lupus patient: case report
Dalmaso,Barbara F.
systemic lupus erythematosus
hemolytic autoimmune anemia
thrombocytopenia
lúpus eritematoso sistêmico
anemia hemolítica autoimune
trombocitopenia
title_short Evans syndrome in male lupus patient: case report
title_full Evans syndrome in male lupus patient: case report
title_fullStr Evans syndrome in male lupus patient: case report
title_full_unstemmed Evans syndrome in male lupus patient: case report
title_sort Evans syndrome in male lupus patient: case report
dc.creator.none.fl_str_mv Dalmaso,Barbara F.
Cortez,Afonso José P.
Velasquez,Maitê Alexandra E.
Silva Neto,Warlindo C.
Silva,Flávio Aparecido R.
Sousa,Lívia Mara S.
author Dalmaso,Barbara F.
author_facet Dalmaso,Barbara F.
Cortez,Afonso José P.
Velasquez,Maitê Alexandra E.
Silva Neto,Warlindo C.
Silva,Flávio Aparecido R.
Sousa,Lívia Mara S.
author_role author
author2 Cortez,Afonso José P.
Velasquez,Maitê Alexandra E.
Silva Neto,Warlindo C.
Silva,Flávio Aparecido R.
Sousa,Lívia Mara S.
author2_role author
author
author
author
author
dc.subject.por.fl_str_mv systemic lupus erythematosus
hemolytic autoimmune anemia
thrombocytopenia
lúpus eritematoso sistêmico
anemia hemolítica autoimune
trombocitopenia
topic systemic lupus erythematosus
hemolytic autoimmune anemia
thrombocytopenia
lúpus eritematoso sistêmico
anemia hemolítica autoimune
trombocitopenia
description ABSTRACT Evans syndrome (ES) is a rare autoimmune disease of unknown etiology. ES occurs when there is a combination of autoimmune hemolytic anemia (AIHA) with thrombocytopenia, whether or not accompanied by immune neutropenia. This syndrome fits into a special variable type of warm AHAI and may be related to rheumatic diseases, such as systemic lupus erythematosus (SLE). Its pathogenesis is still far away to be fully understood. Direct Coombs test results are positive in 98% of cases. ES treatment is required when the anemia due to hemolysis is intense, and corticotherapy is recommended initially. In refractory cases, splenectomy or rituximab therapy, as well as other immunosuppressants, such as azathioprine, may be used. In this study, we present the case of a 45-year-old patient, male, who was diagnosed with ES associated with active SLE. The patient was admitted to the emergency room presenting severe hemolytic anemia and onset of thrombocytopenia. He was treated with intravenous corticosteroid therapy, followed by azathioprine.
publishDate 2019
dc.date.none.fl_str_mv 2019-08-01
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=S1676-24442019000400416
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442019000400416
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.5935/1676-2444.20190038
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.55 n.4 2019
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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