Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening hyperinflammatory syndrome, which requires prompt and aggressive treatment It is termed macrophage activation syndrome (MAS) when associated with rheumatic disease and secondary HLH (sHLH)...
Usually requires ICU management given the severity of clinical features Often first diagnosed in patients with known sepsis or multi-organ failure: Has non-specific symptoms and laboratory findings Requires ICU clinicians to have a high index of suspicion Has specific...
Estimated at <1 per 100,000 children under 18 Likely to be significantly under-recognised Incidence may be as high as 1 in 2000 adult admissions at tertiary medical centres Related Pages: (5.8)...
Primary HLH is almost universally fatal without treatment Mortality improved with treatment Approximately 50% with HLH-94 based treatment Secondary HLH and HLH in adults without treatment has high mortality: Overall mortality of 50% to 75% Stem cell transplant has...
Primary Genetic defects – usually autosomal recessive:Function of cytotoxic T cells or NK cellsInflammasome regulation Secondary (Acquired) Viral InfectionsEBVHSVHIV CMV Other InfectionsBacterialProtozoal (Malaria, Leishmania)Fungal (Candida,...
