is a rare autoimmune disorder in which the body makes antibodies that destroy the red blood cells, platelets and white blood cells. Patients are diagnosed with thrombocytopenia and Coombs' positive hemolytic anemia and have no other known underlying etiology. The patients may be affected by low levels of all three types of blood cells at one time, or may only have problems with one or two of them. The specific cause for Evans syndrome is unknown and it has been speculated that for every case, the cause may be different. There have been no genetic links identified.
The course of Evans syndrome
varies by case. The patient may be symptomatic of whatever blood levels are down. If the red blood cells are down, the problems complained of may be weakness, fatigue, shortness of breath and the usual things associated with anemia. With low platelets, they are susceptible to bleeding and major bruising from minor bumps and cuts. A bump on the head could cause severe brain hemorrhage and death. With low white blood cells, the patient has increased susceptibility to infections and difficulty in fighting these infections. The patient may have problems with one, two or all three of these blood lines, at one time.
Treatment of Evans syndrome
varies and there has been no "magic bullet" identified that will cure this. Steroids are frequently used to help suppress the immune system, or to decrease the production of the "bad antibodies". Intravenous immune globulin or IVIG is often tried as is chemotherapy when responses to other treatments are not satisfactory. Splenectomy has frequently been done, but the benefits of this are usually short-lived. In the last study done, the beneficial effects from splenectomy had only lasted an average of one month. Closely monitoring the patients' complete blood count is crucial to the patients' treatment. Transfusions, of blood products, is done in crisis situations to help stabilize the patient but is not a long lasting solution as these cells are usually destroyed very quickly by the body.
The prognosis with Evans syndrome
is guarded. Some patients have episodes of major blood cell destruction followed by long remissions, while others have chronic problems with no remissions. It has been reported that patients with Evans syndrome have a greater tendency to develop other autoimmune disorders such as lupus and rheumatoid arthritis and there is a tendency to develop various malignancies. Careful monitoring of the patient by a qualified physician is very important.
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