What is Evans syndrome?
The diagnosis of Evans syndrome is made when the patient represents thrombocytopenia and hemolytic anemia that has no other apparent cause. In this disease, the patient's immune system produces antibodies that destroy red and white blood cells and plates. Symptoms vary depending on which type of blood cells is low, but may include weakness, bruises or inability to fight infection. No only treatment is effective in persons with Evans syndrome, but can be tested with steroids, intravenous immunoglobulin (IVIG), rituximab and splenectomy. Some people have a period of active destruction of blood cells and then remission; Others have chronic problems and have a long -term prognosis. In other cases, only one or two components of blood are attacked. It can change from one presentation to another at any time. Syndrome can go in and out of remission, or remain the same, for a lifetime.
The symptoms of Evans syndrome depend on which part of the blood is low. Patients with a low number of redBloods may have fatigue, shortness of breath and weakness. When the plates are low, patients can obtain severe bruises from small bumps around the trunk and limbs and are at risk of bleeding of the brain if they accidentally hit the head. The ability to fight infection decreases if the number of white blood cells is low.
Treatment of a patient with Evans syndrome may be difficult. Steroids to suppress the immune system and reduce the production of antibodies of destroying blood are often the first line of treatment. Prednison is effective for short -term, but patients cannot take this drug indefinitely because potential side effects are too dangerous. Relapses often occur as soon as the patient stops prednisone therapy.
IVIG Top -re -control can be tested if the patient does not respond well to other treatment. Blood plasma collected from multiple donors is given to the patient IV. Its use can help reduce the level of excessively activeImmunity system, helping physical fight against infection and can also neutralize antibodies attacking the system. However, the costs are often unbearable. Side effects such as shortness of breath, fever, heart pain, muscle pain, kidney failure and death may occur.
In Clinical Studies Rituximab, he gave Evans syndrome into remission for 17 months. It is used in patients who have severe symptoms that do not respond to other therapy. The side effects of rituximab are considered minimal.
Splenectomy or removal of the spleen could improve the number of blood, but the way the patient reacts to surgery is a mystery until the procedure is actually performed. The length of remission in patients who have undergone splenectomy rang from one week to five years, with average remission is one month. Children who have removed their spleen tend to have a higher risk of complications after surgery.
patients can go to and out of remission during their lives and their long -term prognosis is protected. They are threatened by the development of other autoimmune diseases such as lupus or rheumatoid arthritis. Those with Evans syndrome must be carefully monitored by a specialist to maximize the length of their lives.