|  | Conditions Treatments | Blood Disorders

Evans Syndrome & Chemotherapy

Evans syndrome, an autoimmune disorder, is the existence of the blood disorders thrombocytopenia and autoimmune hemolytic anemia (AIHA) in the same patient. These two disorders may exist together at the same time, or they may exist sequentially with one or the other entering periods of remission and exacerbation. A third blood disorder, neutropenia, may also affect some patients with Evans syndrome. The underlying cause of the syndrome is unknown. Even its diagnosis is known as one of exclusion, meaning other causes must first be ruled out. Chemotherapy is one treatment option.

  1. Blood Disorders

    • Red blood cells carry oxygen to our cells and cart away carbon dioxide waste products. Anemia is the term used to describe a low red blood cell count, which can have varying causes. AIHA is anemia that results from an autoimmune attack. White blood cells describe a group of different types of immune system cells that fight infection. One such cell is called a neutrophil; a low blood count of neutrophils is known as neutropenia. According to the Mayo Clinic, platelets cause blood clotting; a low platelet count is termed thrombocytopenia.

    Symptoms

    • The symptoms of Evans syndrome depend on which of the blood disorders are occurring in the patient at any given time. AIHA symptoms include fatigue, pale skin color and shortness of breath; in extreme cases jaundice or yellowing of the skin can result. Thrombocytopenia may cause excessive bleeding and bruising; and neutropenia can make a patient more susceptible to bacterial infections. A person with Evans syndrome may experience one or a combination of these symptoms at any given time.

    Disease Course

    • Evans syndrome can affect people of any age, race, location or gender. Some patients may experience chronic, progressive disease during their lifetimes while others will have long periods of remission. Patient response to treatments is variable, although according to eMedicine, patients not receiving treatment generally experience more complications. Occasionally Evans syndrome can be fatal, most often resulting from brain or other internal hemorrhaging due to thrombocytopenia.

    Treatments

    • Medical treatments of Evans syndrome may include blood/platelet transfusions as well as chemotherapy. The term chemotherapy refers to the treatment of disease with chemicals, although in modern culture is often understood to mean anti-cancer drug therapy. For the treatment of Evans syndrome, steroid treatments are considered the first-line treatments, to assist in halting acute attacks and to reduce inflammation. eMedicine also cites intravenous immunoglobulin (IVIG) as an agent used in the treatment of this disease. IVIG is a refined blood product and acts as an immunomodulating agent, meaning that is suppresses the response of the immune system.

    Side Effects

    • As with all medication therapies, the benefit to risk ratios must be considered. Long term prednisone steroid use can have adverse effects such as mood swings, eye problems, body fat redistribution, increased susceptibility to infection and osteoporosis. IVIG therapy's side effect profile includes skin conditions, kidney problems, blood clotting, heart disease and aseptic meningitis, as stated by eMedicine. Ongoing regulation of medication by a physician should be part of the treatment of Evans syndrome, which is a lifelong condition.

Blood Disorders - Related Articles