Clin Infect Pract 2021 Sep 17:100100. Epub 2021 Sep 17.
Professor at Universidade Federal Fluminense, Departamento de Medicina Clínica, Niterói, Rio de Janeiro, Brazil, Hematologist at Hospital Universitário Antônio Pedro, Universidade Federal Fluminense, Niterói, Rio de Janeiro, Brazil.
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by persistent activation of the mononuclear phagocytic system, systemic inflammation, and hypercytokinaemia, which can lead to liver failure, "sepsis-like syndrome" and ultimately, to multiple organ failure and death. These disorders can be divided into primary and secondary (or reactive), the first, also known as familial HLH, is a genetic condition of childhood, which affects the function of TCD8 and NK cells, and usually presents in the first year of life. The secondary HLH affects mainly adults and adolescents and, it's more related to dysregulation of the immune system. Read More