Abstract
Haemophagocytic lymphohistiocytosis (HLH) is a severe condition in which there is extreme uncontrolled inflammation, and may progress rapidly to multi-organ failure and death. HLH may be genetic (primary HLH), or secondary to infection or autoimmune/ autoinflammatory conditions; if the latter, it is also referred to as macrophage activation syndrome (MAS). Distinguishing between primary HLH and MAS is challenging but important since the former requires different therapeutic approaches including allogeneic haematopoietic stem cell transplantation (HSCT) for long-term survival. HLH screening tests are now being used in patients presenting with suspected MAS. In systemic Juvenile Idiopathic Arthritis (sJIA), some patients demonstrate temporary perforin expression abnormalities that resolve with disease control. The utility of other screening tests in a rheumatology context is unknown.
Highlights
Haemophagocytic lymphohistiocytosis (HLH) is a severe condition in which there is extreme uncontrolled inflammation, and may progress rapidly to multi-organ failure and death
HLH screening tests are being used in patients presenting with suspected macrophage activation syndrome (MAS)
In systemic Juvenile Idiopathic Arthritis, some patients demonstrate temporary perforin expression abnormalities that resolve with disease control
Summary
Introduction Haemophagocytic lymphohistiocytosis (HLH) is a severe condition in which there is extreme uncontrolled inflammation, and may progress rapidly to multi-organ failure and death.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have