Infection-associated hemophagocytic syndrome (IAHS), also known as macrophage activation syndrome (MAS), is a rare and potentially life-threatening condition characterized by an overactive immune system response triggered by an infection. It is a severe form of secondary hemophagocytic lymphohistiocytosis (HLH), a disorder where immune cells attack normal tissues.
In IAHS, the body's immune system becomes excessively activated in response to an infection, leading to the release of large amounts of inflammatory substances called cytokines. This immune system hyperactivation leads to widespread inflammation and damage in various organs and tissues, including the liver, spleen, bone marrow, and lymph nodes.
Common symptoms of IAHS include prolonged fever, enlarged organs, low blood cell counts, jaundice, bleeding, anemia, and elevated liver enzymes. If left untreated, the condition can progress rapidly and result in multi-organ failure and death.
To diagnose IAHS, doctors typically perform blood tests to detect abnormalities in cell counts, liver function, and immune markers. Imaging tests, such as X-rays or ultrasounds, may also be performed to assess organ involvement.
Treatment for IAHS involves addressing the underlying infection and managing the hyperinflammatory response. This may include intravenous antibiotics, antiviral medications, or antifungal therapy, depending on the suspected or identified infection. Additionally, medications to suppress the immune system, such as corticosteroids or immunosuppressants, may be given to reduce inflammation. In severe cases, treatments like immune globulin infusions or chemotherapy agents may be necessary to control the immune response.
Overall, IAHS is a serious condition that requires prompt recognition and appropriate management to prevent life-threatening complications.
