Síndrome hemofagocítico secundario: reporte de 5 casos

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 severe hipercytokinemia due to a highly stimulated but ineffective immune system. The principal causes are infections, malignancy or autoimmune disease...

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Autores principales:	Peña,Camila, Valladares,Ximena, Cabrera,María Elena
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2013
Materias:	Lymphohistiocytosis hemophagocytic Lymphoma Pancytopenia
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013001100016
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spelling	oai:scielo:S0034-988720130011000162014-09-01Síndrome hemofagocítico secundario: reporte de 5 casosPeña,CamilaValladares,XimenaCabrera,María Elena Lymphohistiocytosis hemophagocytic Lymphoma Pancytopenia 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 severe hipercytokinemia due to a highly stimulated but ineffective immune system. The principal causes are infections, malignancy or autoimmune diseases. It appears as a serious illness, similar to a multiorgan failure. Treatment is not well defined. We report five patients with HFS, aged 17 to 51 years (three females). The etiology was onco-hematological in three patients. In two patients, the diagnosis was performed during necropsy. One case was due to cytomegalovirus (CMV) infection in a hepatic transplant patient and the other, due to parenteral lipid administration. All presented fever, cytopenia, hepatosplenomegaly and hemophagocytosis. Four of them required admission in an Intensive Care Unit. All received different treatment modalities. Only one survived. Median survival time was 75 days. In conclusion, HFS has different etiologies and a high mortality in adults.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.141 n.11 20132013-11-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013001100016es10.4067/S0034-98872013001100016
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Lymphohistiocytosis hemophagocytic Lymphoma Pancytopenia
spellingShingle	Lymphohistiocytosis hemophagocytic Lymphoma Pancytopenia Peña,Camila Valladares,Ximena Cabrera,María Elena Síndrome hemofagocítico secundario: reporte de 5 casos
description	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 severe hipercytokinemia due to a highly stimulated but ineffective immune system. The principal causes are infections, malignancy or autoimmune diseases. It appears as a serious illness, similar to a multiorgan failure. Treatment is not well defined. We report five patients with HFS, aged 17 to 51 years (three females). The etiology was onco-hematological in three patients. In two patients, the diagnosis was performed during necropsy. One case was due to cytomegalovirus (CMV) infection in a hepatic transplant patient and the other, due to parenteral lipid administration. All presented fever, cytopenia, hepatosplenomegaly and hemophagocytosis. Four of them required admission in an Intensive Care Unit. All received different treatment modalities. Only one survived. Median survival time was 75 days. In conclusion, HFS has different etiologies and a high mortality in adults.
author	Peña,Camila Valladares,Ximena Cabrera,María Elena
author_facet	Peña,Camila Valladares,Ximena Cabrera,María Elena
author_sort	Peña,Camila
title	Síndrome hemofagocítico secundario: reporte de 5 casos
title_short	Síndrome hemofagocítico secundario: reporte de 5 casos
title_full	Síndrome hemofagocítico secundario: reporte de 5 casos
title_fullStr	Síndrome hemofagocítico secundario: reporte de 5 casos
title_full_unstemmed	Síndrome hemofagocítico secundario: reporte de 5 casos
title_sort	síndrome hemofagocítico secundario: reporte de 5 casos
publisher	Sociedad Médica de Santiago
publishDate	2013
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013001100016
work_keys_str_mv	AT penacamila sindromehemofagociticosecundarioreportede5casos AT valladaresximena sindromehemofagociticosecundarioreportede5casos AT cabreramariaelena sindromehemofagociticosecundarioreportede5casos
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Síndrome hemofagocítico secundario: reporte de 5 casos

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