Familial haemophagocytic lymphohistiocytosis (Q88710)

Familial hemophagocytic lymphohistiocytosis (FHL) is characterised by a macrophage activation syndrome presenting with unexplained high fever, irritability, general pain, oedema and hepatosplenomegaly, usually occurring after a healthy period that may range from several months after the birth to, more rarely, several years.