Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases (i.e SLE, Juvenile idiopathic arthritis, Adult onset Still’s disease).
Hemophagocytic lymphohistiocytosis (HLH) is an inherited disease, whereas secondary HLH is 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.
- Lerkvaleekul B, Vilaiyuk S. Macrophage activation syndrome: early diagnosis is key. Open Access Rheumatol. 2018;10:117–128. Published 2018 Aug 31. doi:10.2147/OARRR.S151013