Publicação
Hemophagocytic Syndrome: A Suspect to Have in Mind
| Resumo: | The hemophagocytic syndrome is a rare and potentially fatalcondition, characterized by uncontrolled immune systemactivation, with symptoms and signs of extreme systemic inflammation.As the manifestations are mostly unspecific, itsdiagnosis requires a high degree of suspicion, in order toimplement adequate and timely therapy. The authors presentthe case of a 19 year-old boy, previously healthy, with a suspectedlung infection due to a period of three weeks fever.He presented a sudden increase of liver enzymes and slightpancytopenia, skin rash and hepatosplenomegaly. The elevatedvalues of ferritin and interleukin-2 soluble receptor, aswell as the presence of hemophagocytosis in bone marrow,confirmed the diagnosis of hemophagocytic syndrome. Afterfour weeks of dexamethasone, total resolution of symptomsand laboratorial normalization were achieved. |
|---|---|
| Autores principais: | Souto Moura, Teresa |
| Outros Autores: | Simões, Inês; Lemos, Marcos; Azevedo, Luísa; Gerivaz, Rita; Fonseca, Paula |
| Assunto: | Corticosteroides Ferritinas/sangue Linfohistiocitose Hemofagocítica/diagnóstico Linfohistiocitose Hemofagocítica/ tratamento Adrenal Cortex Hormones Ferritins/blood Lymphohistiocytosis, Hemophagocytic/diagnosis Lymphohistiocytosis, Hemophagocytic/therapy |
| Ano: | 2017 |
| País: | Portugal |
| Tipo de documento: | artigo |
| Tipo de acesso: | unknown |
| Instituição associada: | Sociedade Portuguesa de Medicina Interna |
| Idioma: | português |
| Origem: | Revista Portuguesa de Medicina Interna |
| _version_ | 1869318924871401472 |
|---|---|
| author | Souto Moura, Teresa |
| author2 | Simões, Inês Lemos, Marcos Azevedo, Luísa Gerivaz, Rita Fonseca, Paula |
| author2_role | author author author author author |
| author_facet | Souto Moura, Teresa Simões, Inês Lemos, Marcos Azevedo, Luísa Gerivaz, Rita Fonseca, Paula |
| author_role | author |
| country_str | PT |
| creators_json_txt | [{\"Person.name\":\"Souto Moura, Teresa\"},{\"Person.name\":\"Simões, Inês\"},{\"Person.name\":\"Lemos, Marcos\"},{\"Person.name\":\"Azevedo, Luísa\"},{\"Person.name\":\"Gerivaz, Rita\"},{\"Person.name\":\"Fonseca, Paula\"}] |
| datacite.creators.creator.creatorName.fl_str_mv | Souto Moura, Teresa Simões, Inês Lemos, Marcos Azevedo, Luísa Gerivaz, Rita Fonseca, Paula |
| datacite.rights.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| datacite.subjects.subject.fl_str_mv | Corticosteroides Ferritinas/sangue Linfohistiocitose Hemofagocítica/diagnóstico Linfohistiocitose Hemofagocítica/ tratamento Adrenal Cortex Hormones Ferritins/blood Lymphohistiocytosis, Hemophagocytic/diagnosis Lymphohistiocytosis, Hemophagocytic/therapy |
| datacite.titles.title.fl_str_mv | Hemophagocytic Syndrome: A Suspect to Have in Mind Síndrome Hemofagocítica: Um Suspeito a Considerar |
| dc.creator.none.fl_str_mv | Souto Moura, Teresa Simões, Inês Lemos, Marcos Azevedo, Luísa Gerivaz, Rita Fonseca, Paula |
| dc.description.none.fl_str_mv | A síndrome hemofagocítica é uma entidade rara e potencialmentefatal, caracterizada por uma activação descontroladado sistema imunitário, manifestando-se através de sintomas esinais clínicos e laboratoriais de inflamação sistémica extrema.Pela inespecificidade dos mesmos, o seu diagnóstico requerum elevado grau de suspeição para implementação deterapêutica adequada e atempada. Os autores apresentam ocaso de um jovem de 19 anos, previamente saudável, com febrecom três semanas de evolução, interpretada no contextode infecção respiratória, que subitamente apresentou subidade transaminases e pancitopenia ligeira, exantema cutâneoe hepatoesplenomegália. Os valores elevados de ferritina ereceptor solúvel da interleucina-2, bem como a presença dehemofagocitose na medula óssea vieram confirmar o diagnósticode síndrome hemofagocítica. Após 4 semanas de corticoterapiacom dexametasona, assistiu-se a resolução dossintomas e normalização dos parâmetros laboratoriais. |
| dc.format.none.fl_str_mv | application/pdf |
| dc.identifier.none.fl_str_mv | https://doi.org/10.24950/rspmi.740 |
| dc.language.none.fl_str_mv | por |
| dc.publisher.none.fl_str_mv | Sociedade Portuguesa de Medicina Interna |
| dc.rights.none.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| dc.source.none.fl_str_mv | Medicina Interna; Vol. 24 N.º 2 (2017): Abril/ Junho; 128-132 2183-9980 0872-671X |
| dc.subject.none.fl_str_mv | Corticosteroides Ferritinas/sangue Linfohistiocitose Hemofagocítica/diagnóstico Linfohistiocitose Hemofagocítica/ tratamento Adrenal Cortex Hormones Ferritins/blood Lymphohistiocytosis, Hemophagocytic/diagnosis Lymphohistiocytosis, Hemophagocytic/therapy |
| dc.title.fl_str_mv | Hemophagocytic Syndrome: A Suspect to Have in Mind Síndrome Hemofagocítica: Um Suspeito a Considerar |
| dc.type.none.fl_str_mv | http://purl.org/coar/resource_type/c_6501 http://purl.org/coar/resource_type/c_6501 |
| description | The hemophagocytic syndrome is a rare and potentially fatalcondition, characterized by uncontrolled immune systemactivation, with symptoms and signs of extreme systemic inflammation.As the manifestations are mostly unspecific, itsdiagnosis requires a high degree of suspicion, in order toimplement adequate and timely therapy. The authors presentthe case of a 19 year-old boy, previously healthy, with a suspectedlung infection due to a period of three weeks fever.He presented a sudden increase of liver enzymes and slightpancytopenia, skin rash and hepatosplenomegaly. The elevatedvalues of ferritin and interleukin-2 soluble receptor, aswell as the presence of hemophagocytosis in bone marrow,confirmed the diagnosis of hemophagocytic syndrome. Afterfour weeks of dexamethasone, total resolution of symptomsand laboratorial normalization were achieved. |
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| format | article article |
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| identifier.doi.fl_str_mv | https://doi.org/10.24950/rspmi.740 |
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| instacron_str | SPMI |
| institution | Sociedade Portuguesa de Medicina Interna |
| instname_str | Sociedade Portuguesa de Medicina Interna |
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| organization_str_mv | urn:organizationAcronym:spmi |
| person_str_mv | Souto Moura, Teresa Simões, Inês Lemos, Marcos Azevedo, Luísa Gerivaz, Rita Fonseca, Paula |
| publishDate | 2017 |
| publisher.none.fl_str_mv | Sociedade Portuguesa de Medicina Interna |
| repo_facet_str | urn:repositoryAcronym:spmi{{{_:::_}}}Revista Portuguesa de Medicina Interna |
| reponame_str | Revista Portuguesa de Medicina Interna |
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| spelling | en-USHemophagocytic Syndrome: A Suspect to Have in Mindpt-PTSíndrome Hemofagocítica: Um Suspeito a ConsiderarSouto Moura, TeresaSimões, InêsLemos, MarcosAzevedo, LuísaGerivaz, RitaFonseca, PaulaCorticosteroidesFerritinas/sangueLinfohistiocitose Hemofagocítica/diagnósticoLinfohistiocitose Hemofagocítica/ tratamentoAdrenal Cortex HormonesFerritins/bloodLymphohistiocytosis, Hemophagocytic/diagnosisLymphohistiocytosis, Hemophagocytic/therapyDireitos de Autor (c) 2017 Medicina Internahttp://purl.org/coar/access_right/c_abf2https://creativecommons.org/licenses/by-nc/4.0https://doi.org/10.24950/rspmi.740DOIoai:oai.revista.spmi.pt:article/740OAIhttps://revista.spmi.pt/index.php/rpmi/article/view/740URLhttps://doi.org/10.24950/rspmi.740DOIhttps://revista.spmi.pt/index.php/rpmi/article/view/740/479URLHasVersion2017-06-30T00:00:00Zen-USThe hemophagocytic syndrome is a rare and potentially fatalcondition, characterized by uncontrolled immune systemactivation, with symptoms and signs of extreme systemic inflammation.As the manifestations are mostly unspecific, itsdiagnosis requires a high degree of suspicion, in order toimplement adequate and timely therapy. The authors presentthe case of a 19 year-old boy, previously healthy, with a suspectedlung infection due to a period of three weeks fever.He presented a sudden increase of liver enzymes and slightpancytopenia, skin rash and hepatosplenomegaly. The elevatedvalues of ferritin and interleukin-2 soluble receptor, aswell as the presence of hemophagocytosis in bone marrow,confirmed the diagnosis of hemophagocytic syndrome. Afterfour weeks of dexamethasone, total resolution of symptomsand laboratorial normalization were achieved.pt-PTA síndrome hemofagocítica é uma entidade rara e potencialmentefatal, caracterizada por uma activação descontroladado sistema imunitário, manifestando-se através de sintomas esinais clínicos e laboratoriais de inflamação sistémica extrema.Pela inespecificidade dos mesmos, o seu diagnóstico requerum elevado grau de suspeição para implementação deterapêutica adequada e atempada. Os autores apresentam ocaso de um jovem de 19 anos, previamente saudável, com febrecom três semanas de evolução, interpretada no contextode infecção respiratória, que subitamente apresentou subidade transaminases e pancitopenia ligeira, exantema cutâneoe hepatoesplenomegália. Os valores elevados de ferritina ereceptor solúvel da interleucina-2, bem como a presença dehemofagocitose na medula óssea vieram confirmar o diagnósticode síndrome hemofagocítica. Após 4 semanas de corticoterapiacom dexametasona, assistiu-se a resolução dossintomas e normalização dos parâmetros laboratoriais.Sociedade Portuguesa de Medicina Internaapplication/pdfpt-PTMedicina Interna; Vol. 24 N.º 2 (2017): Abril/ Junho; 128-1322183-99800872-671Xporjournal articlehttp://purl.org/coar/resource_type/c_6501literatureVoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85journal articlehttp://purl.org/coar/resource_type/c_6501literature |
| spellingShingle | Hemophagocytic Syndrome: A Suspect to Have in Mind Souto Moura, Teresa Corticosteroides Ferritinas/sangue Linfohistiocitose Hemofagocítica/diagnóstico Linfohistiocitose Hemofagocítica/ tratamento Adrenal Cortex Hormones Ferritins/blood Lymphohistiocytosis, Hemophagocytic/diagnosis Lymphohistiocytosis, Hemophagocytic/therapy |
| status | SINGLETON |
| status_str | VoR |
| subject.fl_str_mv | Corticosteroides Ferritinas/sangue Linfohistiocitose Hemofagocítica/diagnóstico Linfohistiocitose Hemofagocítica/ tratamento Adrenal Cortex Hormones Ferritins/blood Lymphohistiocytosis, Hemophagocytic/diagnosis Lymphohistiocytosis, Hemophagocytic/therapy |
| title | Hemophagocytic Syndrome: A Suspect to Have in Mind |
| title_full | Hemophagocytic Syndrome: A Suspect to Have in Mind |
| title_fullStr | Hemophagocytic Syndrome: A Suspect to Have in Mind |
| title_full_unstemmed | Hemophagocytic Syndrome: A Suspect to Have in Mind |
| title_short | Hemophagocytic Syndrome: A Suspect to Have in Mind |
| title_sort | Hemophagocytic Syndrome: A Suspect to Have in Mind |
| topic | Corticosteroides Ferritinas/sangue Linfohistiocitose Hemofagocítica/diagnóstico Linfohistiocitose Hemofagocítica/ tratamento Adrenal Cortex Hormones Ferritins/blood Lymphohistiocytosis, Hemophagocytic/diagnosis Lymphohistiocytosis, Hemophagocytic/therapy |
| topic_facet | Corticosteroides Ferritinas/sangue Linfohistiocitose Hemofagocítica/diagnóstico Linfohistiocitose Hemofagocítica/ tratamento Adrenal Cortex Hormones Ferritins/blood Lymphohistiocytosis, Hemophagocytic/diagnosis Lymphohistiocytosis, Hemophagocytic/therapy |
| url | https://doi.org/10.24950/rspmi.740 |
| visible | 1 |