Macrophage activation syndrome
Macrophage Activation Syndrome (pronunciation: mak-roh-fayj ak-ti-vay-shun sin-drohm) is a severe, potentially life-threatening, complication of several rheumatic diseases, which is characterized by the excessive activation and proliferation of macrophages leading to a severe systemic inflammatory response.
Etymology
The term "Macrophage Activation Syndrome" is derived from the Greek words "makros" meaning large, "phagein" meaning to eat, "activation" from the Latin "activare" meaning to set in motion, and "syndrome" from the Greek "syndromos" meaning concurrence of symptoms.
Definition
Macrophage Activation Syndrome (MAS) is a condition characterized by the excessive activation and proliferation of macrophages, a type of white blood cell that engulfs and digests cellular debris, foreign substances, microbes, and cancer cells in a process called phagocytosis. This over-activation results in an extreme inflammatory response and the overproduction of certain chemicals in the body, leading to life-threatening complications.
Symptoms
The symptoms of Macrophage Activation Syndrome can vary, but often include fever, fatigue, rash, lymphadenopathy, hepatosplenomegaly, and coagulopathy.
Causes
Macrophage Activation Syndrome is often associated with certain rheumatic diseases, such as Systemic Juvenile Idiopathic Arthritis (sJIA) and Systemic Lupus Erythematosus (SLE). It can also occur as a result of infections or malignancies.
Diagnosis
Diagnosis of Macrophage Activation Syndrome is based on clinical symptoms, laboratory findings, and the exclusion of other conditions. Laboratory tests often show anemia, thrombocytopenia, hypertriglyceridemia, and elevated levels of ferritin.
Treatment
Treatment for Macrophage Activation Syndrome typically involves high-dose corticosteroids and other immunosuppressive drugs. In severe cases, chemotherapy may be used.
See Also
- Hemophagocytic lymphohistiocytosis
- Cytokine storm
- Rheumatic diseases
- Systemic Juvenile Idiopathic Arthritis
- Systemic Lupus Erythematosus
External links
- Medical encyclopedia article on Macrophage activation syndrome
- Wikipedia's article - Macrophage activation syndrome
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