Evans syndrome is a very rare autoimmune 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: these include the severity of the condition, the signs and symptoms present, and each person's response to certain therapies.
The signs and symptoms of Evans syndrome vary from person to person and largely depend on which type(s) of blood cells are affected (i.e. platelets, white blood cells, or red blood cells). If a person does not have enough healthy red blood cells (anemia), they may experience weakness, fatigue, paleness, light-headedness, and shortness of breath. Low platelets can cause easy or unexplained bruising; prolonged bleeding from small cuts; and purpura. People with low white blood cells may be more susceptible to infections.
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.
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.
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. 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 or splenectomy 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. People undergoing splenectomy may show immediate improvement; however, relapses are common and, in most cases, occur within 1-2 months after the procedure.
For cases that are very severe and difficult to treat, stem cell transplantation may be used to provide a long-term cure. Autologous and allogeneic stem cell transplantation have been used in a small number of patients (14 patients aged 5-52 years), with mixed results.
Those with Evans syndrome rarely do well without treatment. Even with treatment, response to therapy can be 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.variable and often disappointing.
If you or someone you know is battling Evans syndrome, keep fighting HA.R.D.! You are not alone!