Evans syndrome is a very rare autoimmune disorder in which the immune system destroys the body's red blood cells, white blood cells and/or platelets. Affected people often experience thrombocytopenia (too few platelets) and Coombs' positive hemolytic anemia (premature destruction of red blood cells). Signs and symptoms may include purpura, paleness, fatigue, and light-headedness. The exact cause of this condition is unknown. The best treatment options for Evans syndrome depend on many factors, including the severity of the condition; the signs and symptoms present; and each person's response to certain therapies.

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.

If there is a family history of Evans syndrome, we would recommend consulting with a genetics professional to discuss risks for family members.

Many people with Evans syndrome go through periods of remission in which the signs and symptoms of the condition temporarily disappear or become less severe (usually induced temporarily by treatment).

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.

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.

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.