Evans syndrome

The exact cause of Evans syndrome is not known; however, it is known that Evans syndrome is a disorder of the immune system. The immune system is a network of cells, tissues, and organs that work together to defend the body against germs (foreign substances). The immune system normally responds to foreign substances by producing specialized proteins, called antibodies, that target foreign invaders for eventual destruction by white blood cells. Disorders of the immune system like Evans syndrome occur when the immune system produces antibodies that mistakenly attack healthy tissue, specifically red blood cells, platelets, and white blood cells.

Evans syndrome may occur in combination with another disorder as a secondary condition. Disorders that can be associated with Evans syndrome include but are not limited to: autoimmune lymphoproliferative syndrome (ALPS), lupus, antiphospholipid syndrome, Sjogren syndrome, common variable immunodeficiency, IgA deficiency, certain lymphomas, and chronic lymphocytic leukemia.

Evans syndrome is a diagnosis of exclusion. This means that a diagnosis is made in people with Coombs-positive hemolytic anemia and thrombocytopenia related to an abnormal immune response once other conditions with similar signs and symptoms have been ruled out. Various blood tests, and in some cases a bone marrow aspiration, may be needed to exclude other conditions.

Evans syndrome has a chronic, relapsing, and sometimes fatal course. In a 3-year follow-up study of 42 people with Evans syndrome (ages 4 months to 19 years), 3 people (7%) died; 20 people (48%) had active disease and remained on some treatment; and 5 people (12%) had persistent disease but were not receiving any treatment. Fourteen people (33%) had no evidence of disease for 1.5 months to 5 years (median 1 year).

While there is no one cure for Evans syndrome, there are many methods that used to manage symptoms. For some individuals, treatment can lead to long periods of remission in which the signs and symptoms of Evans syndrome are more mild or disappear.

After a thorough review of medical journal articles, we were not able to find evidence of Evans syndrome being contagious.

The long-term outlook for people with Evans syndrome can vary. Some affected people may experience periods of long remission in which the signs and symptoms of the condition disappear or become less severe. Others have chronic problems with no remissions.

Those with Evans syndrome rarely do well without treatment. Even with treatment, response to therapy can be variable and often disappointing. Recurrences of thrombocytopenia and anemia are common, as are episodes of hemorrhage (bleeding) and serious infections. People with Evans syndrome have a greater tendency to develop other autoimmune disorders such as systemic lupus erythematosus (SLE), lymphoproliferative disorders, or primary immunodeficiencies. Evans syndrome is sometimes fatal so careful monitoring by a physician who is familiar with this condition is important.

Treatment for Evans syndrome depends on many factors, including the severity of the condition; the signs and symptoms present; and each person's response to certain therapies. For example, people who need to be hospitalized due to severe anemia or thrombocytopenia are often treated with blood transfusions followed by therapy with corticosteroids or intravenous (IV) immune globulin. Other treatment options include immunosuppressive drugs. Most affected individuals respond to these treatments; however, relapse is frequent.

In people who do not respond to standard treatments, therapy with rituximab may be considered. Some people with Evans syndrome respond well to rituximab treatment and experience an extended period of remission, while others have little to no response.

The role of splenectomy in treating Evans syndrome is not clearly established. While splenectomy may lead to immediate improvement, relapses are common and usually occur within 1-2 months after the procedure. However, occasionally it may result in long-term remission, and there is some evidence that it may help to reduce the frequency of relapses. Because the effectiveness varies and symptoms usually return, splenectomy is usually delayed or avoided as much as possible.

For cases that are very severe and difficult to treat, a stem cell transplant may be used to provide a long-term cure. Autologous and allogeneic stem cell transplantation have been used in a small number of patients with mixed results.