Vol. 5, Issue 2, Part A (2023)

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening hyperinflammatory syndrome characterized by uncontrolled activation of immune cells. It can be categorized into primary and secondary HLH, with the latter often triggered by infections, malignancies, or autoimmune diseases. This abstract provides an overview of HLH, including its clinical features, diagnostic criteria, and treatment approaches. HLH typically presents with fever, hepatosplenomegaly, cytopenias, and hyperferritinemia. Diagnostic criteria include genetic mutations, elevated inflammatory markers, and evidence of hemophagocytosis. Treatment involves immunosuppression, with a combination of chemotherapy, corticosteroids, and hematopoietic stem cell transplantation being the mainstay. Despite advancements in its understanding and management, HLH remains a diagnostic challenge due to its varied presentation and overlapping features with other conditions. Early recognition and timely intervention are crucial in improving outcomes for patients with HLH. Further research is needed to enhance our understanding of the underlying mechanisms and to develop targeted therapies for this complex disorder.