Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literatura

Hemophagocytic syndrome (HS) is a severe hyper inflammatory condition whose cardinal symptoms are prolonged fever, cytopenia, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. The clinical course resembles sepsis, sharing similar physiopathological features....

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Autores principales: YOUNG,PABLO, PERONI,JOSE, FINN,BÁRBARA C, VENDITTI,JULIO E, PREITI,VERÓNICA, BULLORSKY,EDUARDO, BRUETMAN,JULIO E
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2011
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000200013
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spelling oai:scielo:S0034-988720110002000132011-08-01Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literaturaYOUNG,PABLOPERONI,JOSEFINN,BÁRBARA CVENDITTI,JULIO EPREITI,VERÓNICABULLORSKY,EDUARDOBRUETMAN,JULIO E Adrenal cortex hormones Leukopenia Lymphohisiocytosis Hemophagocytic syndrome (HS) is a severe hyper inflammatory condition whose cardinal symptoms are prolonged fever, cytopenia, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. The clinical course resembles sepsis, sharing similar physiopathological features. We report four patients with the syndrome. A 61-year-old female presenting with fever and pleuritic pain. During the course of the disease, a pancytopenia was detected and a bone marrow aspiration was suggestive of HS. The patient was treated with cyclosporine and steroids with a good response. A 61-year-old male with fever and pancytopenia and a bone marrow aspirate suggestive of HS. The patient did not respond to treatment and died. A 23-year-old male with fever, pancytopenia and positive Hanta virus antibodies. A bone marrow aspirate was suggestive of HS. The patient recovered without any treatment. A 72-year-old male admitted with the diagnosis of pneumonia, that developed a progressive pancytopenia and bone marrow aspirate was suggestive of HS. A bronchoalveolar lavage showed the presence of Acinetobacter baumanii. Despite treatment with methylprednisolone and gammaglobulin, the patient died. Awareness of the clinical symptoms and of the diagnostic criteria of HS is important to start life-saving therapy in time.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.139 n.2 20112011-02-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000200013es10.4067/S0034-98872011000200013
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Adrenal cortex hormones
Leukopenia
Lymphohisiocytosis
spellingShingle Adrenal cortex hormones
Leukopenia
Lymphohisiocytosis
YOUNG,PABLO
PERONI,JOSE
FINN,BÁRBARA C
VENDITTI,JULIO E
PREITI,VERÓNICA
BULLORSKY,EDUARDO
BRUETMAN,JULIO E
Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literatura
description Hemophagocytic syndrome (HS) is a severe hyper inflammatory condition whose cardinal symptoms are prolonged fever, cytopenia, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. The clinical course resembles sepsis, sharing similar physiopathological features. We report four patients with the syndrome. A 61-year-old female presenting with fever and pleuritic pain. During the course of the disease, a pancytopenia was detected and a bone marrow aspiration was suggestive of HS. The patient was treated with cyclosporine and steroids with a good response. A 61-year-old male with fever and pancytopenia and a bone marrow aspirate suggestive of HS. The patient did not respond to treatment and died. A 23-year-old male with fever, pancytopenia and positive Hanta virus antibodies. A bone marrow aspirate was suggestive of HS. The patient recovered without any treatment. A 72-year-old male admitted with the diagnosis of pneumonia, that developed a progressive pancytopenia and bone marrow aspirate was suggestive of HS. A bronchoalveolar lavage showed the presence of Acinetobacter baumanii. Despite treatment with methylprednisolone and gammaglobulin, the patient died. Awareness of the clinical symptoms and of the diagnostic criteria of HS is important to start life-saving therapy in time.
author YOUNG,PABLO
PERONI,JOSE
FINN,BÁRBARA C
VENDITTI,JULIO E
PREITI,VERÓNICA
BULLORSKY,EDUARDO
BRUETMAN,JULIO E
author_facet YOUNG,PABLO
PERONI,JOSE
FINN,BÁRBARA C
VENDITTI,JULIO E
PREITI,VERÓNICA
BULLORSKY,EDUARDO
BRUETMAN,JULIO E
author_sort YOUNG,PABLO
title Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literatura
title_short Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literatura
title_full Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literatura
title_fullStr Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literatura
title_full_unstemmed Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literatura
title_sort síndrome hematofagocítico: reporte de cuatro casos y revisión de la literatura
publisher Sociedad Médica de Santiago
publishDate 2011
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000200013
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