Hemophagocytic lymph histiocytosis (HLH): etiologies, pathogenesis, treatment, and outcomes in critically ill patients: a review article and literature to review

Abstract

Hemophagocytic lymph histiocytosis (HLH) is not an independent disease but is instead a clinical syndrome that occurs in many underlying conditions involving all age groups. HLH is the consequence of a severe, uncontrolled hyperinflammatory reaction that in most cases is triggered by an infectious agent. Acquired HLH is much more common than primary HLH syndrome but primary is more fatal, and it does have the worst prognosis with no definitive treatment available to date. This review article mentioned all the latest advancements regarding etiologies, pathogenesis, treatment, and outcomes in critically ill patients who got diagnosed with HLH syndrome in last 15 years.

Síndromes hemofagocíticos vs. linfohistiocitosis hemofagocítica

“…La LHH debe ser sospechada ante todo paciente con una desproporcionada respuesta inflamatoria: fiebre persistente que no responde a vasopresores, necesidad de oxigenación con membrana extracorpórea, fallo multiorgánico e inexplicables citopenias que no responden al tratamiento enérgico 17 . Aunque aquí aplican todas las consideraciones ya comentadas, cabe señalar el valor discriminativo que representan los niveles de ferritina muy elevados en al caso de la LHH en comparación con la sepsis, la utilidad del uso de anakinra y el incremento de la mortalidad que parece conllevar el uso de ciclosporina en las LHH desencadenadas por infecciones 25 .…”

Hemophagocytic syndromes vs. hemophagocytic lymphohistiocytosis

“…HLH should be suspected in any patient with a disproportionate inflammatory response: persistent fever unresponsive to vasopressors, need for extracorporeal membrane oxygenation, multiple organ failure, and unexplained cytopenias unresponsive to aggressive treatment 17 . While all the considerations mentioned earlier apply here, we should mention the discriminatory value of very high ferritin levels compared to sepsis, the utility of using anakinra, and the higher mortality rate associated with the use of cyclosporine in HLH triggered by infections 25 .…”