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. |
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| 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 |
| 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. |
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