Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult

Detalhes bibliográficos
Autor(a) principal: Costa, Andreia Sofia
Data de Publicação: 2019
Outros Autores: Paixão, Anusca, Santos, Henrique, Salvador, Fernando
Tipo de documento: Artigo
Idioma: por
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474
Resumo: Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome, characterized by an excessive immune activation. It is triggered by multiple stimuli, with infections having an important role. The most common infectious trigger is viral infection, particularly by Epstein-Barr virus. Coagulation disorders are common in hemophagocytic lymphohistiocytosis and disseminated intravascular coagulation can be present in severe cases. We report a clinical case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, complicated with disseminated intravascular coagulation which evolved favorably with only supportive therapy and without specific treatment.
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spelling Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent AdultLinfohistiocitose Hemofagocítica Secundária a Infeção por Epstein-Barr: Raridade e Gravidade num Adulto ImunocompetenteDisseminated Intravascular CoagulationEpstein-Barr Virus InfectionsImmunocompromised HostLymphohistiocytosisHemophagocyticShockSepticChoque SépticoCoagulação Intravascular DisseminadaHospedeiro ImunocomprometidoInfecções por Vírus Epstein-BarrLinfohistiocitose HemofagocíticaHemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome, characterized by an excessive immune activation. It is triggered by multiple stimuli, with infections having an important role. The most common infectious trigger is viral infection, particularly by Epstein-Barr virus. Coagulation disorders are common in hemophagocytic lymphohistiocytosis and disseminated intravascular coagulation can be present in severe cases. We report a clinical case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, complicated with disseminated intravascular coagulation which evolved favorably with only supportive therapy and without specific treatment.A linfohistiocitose hemofagocítica é uma entidade clínica rara, agressiva e que pode colocar em risco a vida do doente, caraterizada por uma ativação imune excessiva. É desencadeada por vários estímulos, em que as infeções desempenham um papel importante. O estímulo infecioso mais comum é a infeção viral, especialmente por Epstein-Barr. As alterações da coagulação são comuns na linfohistiocitose hemofagocítica e a coagulação intravascular disseminada pode estar presente nos casos graves. Apresentamos um caso clínico de linfohistiocitose hemofagocítica secundária a infeção por Epstein-Barr, complicada com coagulação intravascular disseminada, que evoluiu favoravelmente apenas com tratamento de suporte e sem necessidade de tratamento específico.Ordem dos Médicos2019-02-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfapplication/pdfimage/jpegimage/jpegapplication/pdfapplication/pdfapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474oai:ojs.www.actamedicaportuguesa.com:article/9474Acta Médica Portuguesa; Vol. 32 No. 1 (2019): January; 78-80Acta Médica Portuguesa; Vol. 32 N.º 1 (2019): Janeiro; 78-801646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/5594https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/9529https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/9844https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/9845https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/9856https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/10049https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/10750Direitos de Autor (c) 2019 Acta Médica Portuguesainfo:eu-repo/semantics/openAccessCosta, Andreia SofiaPaixão, AnuscaSantos, HenriqueSalvador, Fernando2022-12-20T11:05:46Zoai:ojs.www.actamedicaportuguesa.com:article/9474Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:19:43.437448Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult
Linfohistiocitose Hemofagocítica Secundária a Infeção por Epstein-Barr: Raridade e Gravidade num Adulto Imunocompetente
title Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult
spellingShingle Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult
Costa, Andreia Sofia
Disseminated Intravascular Coagulation
Epstein-Barr Virus Infections
Immunocompromised Host
Lymphohistiocytosis
Hemophagocytic
Shock
Septic
Choque Séptico
Coagulação Intravascular Disseminada
Hospedeiro Imunocomprometido
Infecções por Vírus Epstein-Barr
Linfohistiocitose Hemofagocítica
title_short Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult
title_full Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult
title_fullStr Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult
title_full_unstemmed Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult
title_sort Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult
author Costa, Andreia Sofia
author_facet Costa, Andreia Sofia
Paixão, Anusca
Santos, Henrique
Salvador, Fernando
author_role author
author2 Paixão, Anusca
Santos, Henrique
Salvador, Fernando
author2_role author
author
author
dc.contributor.author.fl_str_mv Costa, Andreia Sofia
Paixão, Anusca
Santos, Henrique
Salvador, Fernando
dc.subject.por.fl_str_mv Disseminated Intravascular Coagulation
Epstein-Barr Virus Infections
Immunocompromised Host
Lymphohistiocytosis
Hemophagocytic
Shock
Septic
Choque Séptico
Coagulação Intravascular Disseminada
Hospedeiro Imunocomprometido
Infecções por Vírus Epstein-Barr
Linfohistiocitose Hemofagocítica
topic Disseminated Intravascular Coagulation
Epstein-Barr Virus Infections
Immunocompromised Host
Lymphohistiocytosis
Hemophagocytic
Shock
Septic
Choque Séptico
Coagulação Intravascular Disseminada
Hospedeiro Imunocomprometido
Infecções por Vírus Epstein-Barr
Linfohistiocitose Hemofagocítica
description Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome, characterized by an excessive immune activation. It is triggered by multiple stimuli, with infections having an important role. The most common infectious trigger is viral infection, particularly by Epstein-Barr virus. Coagulation disorders are common in hemophagocytic lymphohistiocytosis and disseminated intravascular coagulation can be present in severe cases. We report a clinical case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, complicated with disseminated intravascular coagulation which evolved favorably with only supportive therapy and without specific treatment.
publishDate 2019
dc.date.none.fl_str_mv 2019-02-01
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dc.type.driver.fl_str_mv info:eu-repo/semantics/article
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/9529
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/9844
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/9856
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/10049
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/9474/10750
dc.rights.driver.fl_str_mv Direitos de Autor (c) 2019 Acta Médica Portuguesa
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Direitos de Autor (c) 2019 Acta Médica Portuguesa
eu_rights_str_mv openAccess
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dc.publisher.none.fl_str_mv Ordem dos Médicos
publisher.none.fl_str_mv Ordem dos Médicos
dc.source.none.fl_str_mv Acta Médica Portuguesa; Vol. 32 No. 1 (2019): January; 78-80
Acta Médica Portuguesa; Vol. 32 N.º 1 (2019): Janeiro; 78-80
1646-0758
0870-399X
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