Hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis is a rare, life-threatening hyperinflammatory syndrome characterized by severe immune dysregulation and uncontrolled activation of cytotoxic T lymphocytes, natural killer cells, and macrophages Pathophysiologically, the failure of normal cytotoxic down-regulation leads to hypercytokinemia, excessive tissue infiltration, and hemophagocytosis across the reticuloendothelial and hematologic systems Hallmark clinical features include persistent high fevers, hepatosp...

Hemophagocytic lymphohistiocytosis is a rare, life-threatening hyperinflammatory syndrome characterized by severe immune dysregulation and uncontrolled activation of cytotoxic T lymphocytes, natural killer cells, and macrophages The condition can affect individuals of any age, presenting either as a primary inherited disorder typically emerging in infancy or early childhood, or as an acquired secondary syndrome in older children and adults triggered by severe infections, malignancies, or autoimm...

The condition can affect individuals of any age, presenting either as a primary inherited disorder typically emerging in infancy or early childhood, or as an acquired secondary syndrome in older children and adults triggered by severe infections, malignancies, or autoimmune diseases Hallmark clinical features include persistent high fevers, hepatosplenomegaly, progressive cytopenias, marked hyperferritinemia, hypofibrinogenemia, and rapid progression toward multiorgan dysfunction

Pathophysiologically, the failure of normal cytotoxic down-regulation leads to hypercytokinemia, excessive tissue infiltration, and hemophagocytosis across the reticuloendothelial and hematologic systems