Evan's syndrome is an extremely rare autoimmune disorder. The immune system destroys the body's red blood cells, platelets, and/or white blood cells.
Those who suffer from this illness may have the following:
-Thrombocytopenia: Lack of platelets in the body.
-Coombs' positive hemolytic anemia: premature destruction of the red blood cells.
What causes Evan's syndrome?
Exact causes are unknown, but researchers believe that the syndrome is a dysregulation of the immune system.
What are the symptoms of Evan's syndrome?
Symptoms vary from person to person, as with any disease. Symptoms may vary according to what blood cells may be affected. Some of the most common symptoms associated with this syndrome include:
-Shortness of breath
-Prolonged bleeding from small cuts
-Susceptibility to infections
Individuals with Evan's syndrome may go through periods of remission, where the symptoms become less severe.
How is Evan's syndrome diagnosed?
Your doctor will be checking your symptoms and sending you for blood tests. In some cases, your doctor may order a bone marrow aspiration procedure in order to make a definitive diagnosis.
How is Evan's syndrome treated?
Treatment depends on your symptoms and severity of your condition.
Those who have severe anemia may be hospitalized and treated with blood transfusions. Afterward, patients will be treated with corticosteroids and IV immune globulin. Immunosuppressive drugs may also be considered as a form of treatment.
Those who do not respond to these treatments may be recommended for rituximab or splenectomy therapies.
Those who have a very severe case of Evan's syndrome may be recommended for stem cell transplantation.
It is important for those living with Evan's syndrome to be carefully monitored by their doctor. While you may go through periods of remission, going without medication, and not communicating with your doctor, can put you at serious risk. Evan's syndrome has a history of being a fatal disease.
Keep in contact with your doctor, and never discontinue your medication UNLESS your doctor says so.
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