Understanding Hemophagocytic Lymphohistiocytosis (HLH)

HLH is a complex disease characterized by excessive immune activation and inability to terminate the immune response. Patients with HLH experience hyperinflammation due to unchecked cytokine production, leading to multi-organ failure and severe clinical manifestations like fever, hypotension, and organ failure. Prompt recognition of HLH is vital to prevent adverse outcomes, as delays can result in high mortality rates. The disease is primarily classified into primary (familial) and secondary HLH, with primary HLH linked to genetic mutations affecting immune function.