Síndrome hemofagocítico secundario: reporte de 5 casos / Secondary hemophagocytic syndrome: report of 5 cases
Rev. méd. Chile
;
141(11): 1475-1479, nov. 2013. ilus, tab
Artigo
em Espanhol
| LILACS
| ID: lil-704574
ABSTRACT
Secondary hemophagocytic syndrome (HFS) is an uncommon entity with a high mortality rate in adults, if no therapy is given. It is characterized by a severehipercytokinemia due to a highly stimulated but ineffective immune system. Theprincipal causes are infections, malignancy or autoimmune diseases. It appears asa serious illness, similar to a multiorgan failure. Treatment is not well defined. Wereport five patients with HFS, aged 17 to 51 years (three females). The etiology wasonco-hematological in three patients. In two patients, the diagnosis was performedduring necropsy. One case was due to cytomegalovirus (CMV) infection in a hepatictransplant patient and the other, due to parenteral lipid administration. All presentedfever, cytopenia, hepatosplenomegaly and hemophagocytosis. Four of them requiredadmission in an Intensive Care Unit. All received different treatment modalities. Onlyone survived. Median survival time was 75 days. In conclusion, HFS has differentetiologies and a high mortality in adults.
Texto completo:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Infecções por Citomegalovirus
/
Linfo-Histiocitose Hemofagocítica
/
Soluções de Nutrição Parenteral
/
Lipídeos
/
Linfoma
Limite:
Adolescente
/
Adulto
/
Feminino
/
Humanos
/
Masculino
Idioma:
Espanhol
Revista:
Rev. méd. Chile
Assunto da revista:
Medicina
Ano de publicação:
2013
Tipo de documento:
Artigo
País de afiliação:
Chile
Instituição/País de afiliação:
Hospital del Salvador/CL
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