Background Over the last few years, accumulating data have implicated a role for ferritin as
a signaling molecule and direct mediator of the immune system. Hyperferritinemia is …

Hemophagocytic lymphohistiocytosis (HLH) is not an independent disease but rather a life-
threatening clinical syndrome that occurs in many underlying conditions and in all age …

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory
syndrome and not an independent disease. HLH represents the extreme end of a severe …

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory
syndrome requiring aggressive immunosuppressive therapy. Following 2 large international …

Most knowledge of hemophagocytic syndromes (HPSs) including hemophagocytic
lymphohistiocytosis (HLH) is derived from pediatric studies; literature on adult HPS/HLH …

To investigate the clinical features of adult patients with hemophagocytic lymphohistiocytosis
(HLH) and to explore possible risk factors for death, we retrospectively reviewed the medical …

Haemophagocytic lymphohistiocytosis (HLH) comprises a heterogeneous spectrum of
hyperinflammatory conditions that are inherited (primary HLH) or acquired in a context of …

Macrophage activation syndrome (MAS), also known as secondary hemophagocytic
lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD) …

Macrophage activation syndrome (MAS) is an episode of overwhelming inflammation that
occurs most commonly in children with systemic juvenile idiopathic arthritis (SJIA). It is …

Highlights•HLH is a life-threatening hyperinflammatory syndrome.•Genetic defects impairing
lymphocyte cytotoxicity mainly cause primary HLH.•Defects primary impairing macrophages …