Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases (i.e SLE, Juvenile idiopathic arthritis, Adult onset Still’s disease). It manifests as uncontrolled activation and proliferation of T lymphocytes and macrophages
Hemophagocytic lymphohistiocytosis (HLH) is an inherited disease, or secondary overactivation of histiocytes and lymphocytes triggered by other diseases, including infections (i.e EBV, HSV, CMV), malignancy (i.e Lymphoma, Leukemia), and autoimmune diseases.
***Both can present with high-grade fever (>39C), hemorrhagic manifestations (DIC), hepatosplenomegaly and lymphadenopathy.
BOTH require immediate and appropriate treatment.
REFERENCES
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