macrophage activating syndrome (mas) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, still's disease, polyarteritis nodosa. it is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. a 27 yr. old female patient was admitted in shahid mostafa khomeini hospital (tehra...

Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Ira...

PURPOSE OF THE REVIEW One of the most perplexing features of systemic-onset juvenile rheumatoid arthritis is the association with macrophage activation syndrome, a life-threatening complication caused by excessive activation and proliferation of T cells and macrophages. The main purpose of the review is to summarize current understanding of the relation between macrophage activation syndrome an...

Macrophage activation syndrome (MAS) is a subtype of hemophagocytic lymphohistiocytosis (HLH) diseases. The underlying mechanism these life-threatening disorders impaired granule-mediated cytotoxicity exerted by natural killer (NK) cells and T lymphocytes. This function meant for elimination virus-infected cells, malignant to prevent exaggerated immune responses. normal outcome after an attack ...

Macrophage activation syndrome is a form of secondary hemophagocytic lymphohistiocytosis in the setting autoimmune diseases. It’s rare, hyperinflammatory complication requiring prompt institution therapy to prevent organ dysfunction and death. We report young female presenting with macrophage multiorgan failure diffuse alveolar hemorrhage inaugural systemic lupus erythematosus. The patient was ...

Hemophagocytic syndrome is a macrophage disorder that may develop as a result of immunological activation, such as that seen in severe infection. It has been described in persons with HIV/AIDS and in those with a variety of HIV-associated opportunistic infections, including those caused by Histoplasma capsulatum. If present, this disorder may portend a poorer prognosis. We describe an HIV-posit...

1 Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome (HPS). Autoimmun Rev 2004;3:69–75. 2 Ravelli A. Macrophage activation syndrome. Curr Opin Rheumatol 2002;14: 548–52. 3 Hibi S, Ikushima S, Fujiwara F et al. Serum and urine beta-2-microglobulin in hemophagocytic syndrome. Cancer 1995;75:1700–5. 4 Wellwood JM, Ellis BG, Price RG, Hammond K, Thompson AE, Jones NF. Urinary N-acetylbe...

1 Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome (HPS). Autoimmun Rev 2004;3:69–75. 2 Ravelli A. Macrophage activation syndrome. Curr Opin Rheumatol 2002;14: 548–52. 3 Hibi S, Ikushima S, Fujiwara F et al. Serum and urine beta-2-microglobulin in hemophagocytic syndrome. Cancer 1995;75:1700–5. 4 Wellwood JM, Ellis BG, Price RG, Hammond K, Thompson AE, Jones NF. Urinary N-acetylbe...

Macrophage activation syndrome (MAS) is a rare feature of rheumatic disorders in children and adolescence and its presentation as the first symptom of rheumatic disorders is very infrequent. A 9-year-old girl, in whom MAS developed, was admitted to our Hospital in Tehran, Iran. She suffered from high grade fever and rash followed by multiple joint swelling months afterwards. Bone marrow aspira...