Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr

Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a highly lethal hyper-inflammatory disorder that leads to a storm of cytokines, hemophagocytosis and multiple organ failure. It can be primary, which is inherited, or secondary. In the latter, virus infections are a frequent trig...

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Autores principales: Otárola B.,Daniela, Troncoso L.,Nicolás, Álvarez C.,Daniel, Bahamondes M.,Laura
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2020
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872020000300371
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spelling oai:scielo:S0034-988720200003003712020-06-11Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-BarrOtárola B.,DanielaTroncoso L.,NicolásÁlvarez C.,DanielBahamondes M.,Laura Diagnosis, Treatment Hemophagocytic Herpesvirus 4, Human Lymphohistiocytosis, Macrophage Activation Syndrome Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a highly lethal hyper-inflammatory disorder that leads to a storm of cytokines, hemophagocytosis and multiple organ failure. It can be primary, which is inherited, or secondary. In the latter, virus infections are a frequent trigger, predominantly the family of herpes viruses, such as Epstein-Barr virus. An early treatment is recommended. Until recently there was no consensus about the management of secondary cases. The protocols for the treatment of primary HLH were used, which include cytotoxic agents and corticosteroids. We herein review the current diagnostic and therapeutic approach of HLH, based on a case associated with a reactivation of the Epstein-Barr virus in an immunocompetent adolescent. We highlight the importance of suspecting this disease in patients with a persistent inflammatory response state or with a fever of unknown origin, in order to carry out a timely treatment, with the least toxicity, and appropriate to the characteristics of each individual, which is the current therapeutic trend.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.148 n.3 20202020-03-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872020000300371es10.4067/S0034-98872020000300371
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Diagnosis, Treatment
Hemophagocytic
Herpesvirus 4, Human
Lymphohistiocytosis, Macrophage Activation Syndrome
spellingShingle Diagnosis, Treatment
Hemophagocytic
Herpesvirus 4, Human
Lymphohistiocytosis, Macrophage Activation Syndrome
Otárola B.,Daniela
Troncoso L.,Nicolás
Álvarez C.,Daniel
Bahamondes M.,Laura
Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr
description Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a highly lethal hyper-inflammatory disorder that leads to a storm of cytokines, hemophagocytosis and multiple organ failure. It can be primary, which is inherited, or secondary. In the latter, virus infections are a frequent trigger, predominantly the family of herpes viruses, such as Epstein-Barr virus. An early treatment is recommended. Until recently there was no consensus about the management of secondary cases. The protocols for the treatment of primary HLH were used, which include cytotoxic agents and corticosteroids. We herein review the current diagnostic and therapeutic approach of HLH, based on a case associated with a reactivation of the Epstein-Barr virus in an immunocompetent adolescent. We highlight the importance of suspecting this disease in patients with a persistent inflammatory response state or with a fever of unknown origin, in order to carry out a timely treatment, with the least toxicity, and appropriate to the characteristics of each individual, which is the current therapeutic trend.
author Otárola B.,Daniela
Troncoso L.,Nicolás
Álvarez C.,Daniel
Bahamondes M.,Laura
author_facet Otárola B.,Daniela
Troncoso L.,Nicolás
Álvarez C.,Daniel
Bahamondes M.,Laura
author_sort Otárola B.,Daniela
title Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr
title_short Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr
title_full Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr
title_fullStr Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr
title_full_unstemmed Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr
title_sort síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. revisión a partir de un caso relacionado a virus epstein-barr
publisher Sociedad Médica de Santiago
publishDate 2020
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872020000300371
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