Macrophage activation syndrome

Macrophage activation syndrome is a life-threatening, hyperinflammatory state categorized as a secondary form of hemophagocytic lymphohistiocytosis

Macrophage activation syndrome is a life-threatening, hyperinflammatory state categorized as a secondary form of hemophagocytic lymphohistiocytosis Pathophysiologically, it is driven by defective cytotoxic cell function and the uncontrolled activation and expansion of T lymphocytes and macrophages, producing a massive cytokine storm and widespread hemophagocytosis

It predominantly complicates pediatric and adult autoimmune or autoinflammatory conditions, most notably systemic juvenile idiopathic arthritis, Adult-onset Still's disease, and systemic lupus erythematosus Clinically, the syndrome manifests as persistent high fevers, hepatosplenomegaly, lymphadenopathy, cytopenias, marked hyperferritinemia, coagulopathy, hepatic dysfunction, and potential multiorgan failure

It predominantly complicates pediatric and adult autoimmune or autoinflammatory conditions, most notably systemic juvenile idiopathic arthritis, Adult-onset Still's disease, and systemic lupus erythematosus

It predominantly complicates pediatric and adult autoimmune or autoinflammatory conditions, most notably systemic juvenile idiopathic arthritis, Adult-onset Still's disease, and systemic lupus erythematosus

Pathophysiologically, it is driven by defective cytotoxic cell function and the uncontrolled activation and expansion of T lymphocytes and macrophages, producing a massive cytokine storm and widespread hemophagocytosis Clinically, the syndrome manifests as persistent high fevers, hepatosplenomegaly, lymphadenopathy, cytopenias, marked hyperferritinemia, coagulopathy, hepatic dysfunction, and potential multiorgan failure

Clinically, the syndrome manifests as persistent high fevers, hepatosplenomegaly, lymphadenopathy, cytopenias, marked hyperferritinemia, coagulopathy, hepatic dysfunction, and potential multiorgan failure