Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr / Hemophagocytic syndrome, current diagnostic and therapeutic approach
Rev. méd. Chile
;
148(3): 371-380, mar. 2020. tab, graf
Article
in Spanish
| LILACS
| ID: biblio-1115801
ABSTRACT
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Lymphohistiocytosis, Hemophagocytic
Type of study:
Diagnostic study
/
Practice guideline
Limits:
Humans
Language:
Spanish
Journal:
Rev. méd. Chile
Journal subject:
Medicine
Year:
2020
Type:
Article
Affiliation country:
Chile
Institution/Affiliation country:
Unidad de Tratamiento Intensivo/CL
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