Haemophagocytic syndromes – macrophages with ‘the munchies’

Haemophagocytic syndrome is a severe hyperinflammatory syndrome with high risk of mortality. It occurs both as a primary condition resulting from genetic defects of NK and T cell function and also as an acquired condition in the setting of infection, rheumato-logical disorders, immunosuppressive therapy and lymphoid malignancies. Whilst presence of haemophagocytosis is a useful finding in some cases it is important to note that it is a clinical syndrome based on the presence of specific symptoms, signs and abnormalities of laboratory testing. However diagnosis is often delayed or missed due to the overlap of these with other severe and fulminating conditions, particularly in the settings of suspected sepsis or liver failure due unknown cause. A high level of clinical suspicion is important for early detection and appropriate treatment.