What is Evans Syndrome?

The diagnosis of Evans syndrome is made when a patient presents with thrombocytopenia and hemolytic anemia that have no other apparent cause. In this disease, the patient's immune system produces antibodies that destroy red and white blood cells and platelets. Symptoms vary depending on which type of blood cell is low, but may include weakness, bruising, or an inability to fight off infection. No single treatment is effective for those with Evans syndrome, but steroids, intravenous immunoglobulin (IVIG), rituximab, and splenectomy may be tried. Some people have periods of active blood cell destruction followed by remission; others experience chronic problems and have a guarded long-term prognosis.

The disease, which does not appear to be genetic, sometimes destroys all three blood components at the same time. In other cases, only one or two of the blood components are under attack. It may change from one presentation to another at any time. The syndrome may go in and out of remission, or stay the same, throughout life.

Symptoms of Evans syndrome depend on which part of the blood is low. Patients with low red blood cell count may experience fatigue, shortness of breath, and weakness. When platelets are low, patients may get severe bruises from small bumps to the trunk and extremities and are at risk of a brain hemorrhage if they accidentally bump their head. The ability to fight infection diminishes if the white blood cell count is low.

Treating a patient with Evans syndrome may be difficult. Steroids to suppress the immune system and reduce the production of blood-destroying antibodies are often the first line of treatment. Prednisone is effective for the short-term, but patients cannot take this medication indefinitely because potential side effects are too dangerous. Relapses frequently occur as soon as the patient stops prednisone therapy.

IVIG treatment may be tried if the patient does not respond well to other treatments. Blood plasma collected from multiple donors is given to the patient by IV. Its use may help reduce the level of an over-active immune system, help the body fight infection, and may also neutralize the antibodies attacking the system. Cost is often prohibitive, however. Side effects, such as shortness of breath, fever, heart pain, muscle aches, renal failure and death, may occur.

In clinical trials, rituximab has put Evans syndrome into remission for as long as 17 months. It is used with patients who have severe symptoms that are unresponsive to other therapy. Side effects of rituximab are said to be minimal.

Splenectomy, or removal of the spleen, might improve blood counts, but how the patient responds to the surgery is a mystery until the procedure is actually performed. The length of remission for patients who have undergone a splenectomy rang from one week to five years, with the average remission being one month. Children who have their spleens removed tend to have a higher risk of complications following surgery.

Patients may go into and out of remission during their lives, and their long-term prognosis is guarded. They are at risk for developing other autoimmune diseases such as lupus or rheumatoid arthritis. Those with Evans syndrome must be monitored closely by a specialist to maximize the length of their lives.