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.
Highlights
Conflictos de interés: los autores de este artículo declaran no tener conflictos de interés
Los estudios genéticos y funcionales no están recomendados en adultos porque rara vez se detectan las anormalidades[34].Una herramienta de utilidad para el diagnóstico es el “Hscore”[35], que estima el riesgo de tener un síndrome hemofagocítico (SHF) reactivo basado en variables clínicas, biológicas y citológicas
Un estudio retrospectivo en adultos con SHF de causa desconocida en recidiva, mostró que tras ser sometidos a esplenectomía los pacientes tuvieron una sobrevida promedio de 57% a 12 meses y 25% a los 36 meses sugiriendo que podría tener un rol terapéutico en este grupo[54]
Summary
Enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus. En el HLH-94, el primer estudio prospectivo internacional de SHF, el diagnóstico se basaba en 5 criterios: fiebre, esplenomegalia, bicitopenia, hipertrigliceridemia y/o hipofibrinogenemia, y hemofagocitosis. En el estudio HLH-2004 se agregaron 3 criterios: actividad baja o ausente de las células NK, hiperferritinemia y altos niveles del receptor soluble de interleuquina 2 (sCD25). La actividad de las células NK es parte de los criterios diagnósticos para el SHF pediátrico, pero rara vez es útil en la población adulta[27]. Los estudios genéticos y funcionales no están recomendados en adultos porque rara vez se detectan las anormalidades[34].Una herramienta de utilidad para el diagnóstico es el “Hscore”[35], que estima el riesgo de tener un SHF reactivo basado en variables clínicas, biológicas y citológicas (disponible en http://saintantoine.aphp.fr/score/)
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