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What

Autoimmune hemolytic anemia is a condition caused by the immune system which affects the body’s blood. In this condition, there are low levels of iron in the body due to the destruction of the red blood cells (RBC) that normally carry oxygen to the body’s tissues.

Who

Approximately half of all cases of autoimmune hemolytic anemia have unknown causes. In children 2-12 years of age, the disease is much more likely to be acute (short lived, i.e. 3-6 months). The disorder is preceded by a cold or flu in many cases, and this vial infection is thought to trigger the disorder. In infants, older children and adults, autoimmune hemolytic anemia is often a chronic condition, lasting months to years.

Signs and Symptoms

In children with autoimmune hemolytic anemia any of the following signs and symptoms may be found: Fatigue Pale skin color Yellow skin color (jaundice) Shortness of breath Rapid heartbeat Dark urine The following laboratory study results may be found: A positive Coomb’s test. This is a test for antibodies against RBCs. Increased levels of bilirubin levels. bilirubin is the by-product of the destruction of old RBCs. With increased RBC destruction, there will be increased levels of bilirubin. The increased bilirubin is the cause of the yellowish skin color referred to as jaundice, and the dark urine, which is due to bilirubin breakdown products in the urine. Elevated levels of reticulocytes. Reticulocytes are the precursors to RBCs. The levels are increased because the body is trying to rapidly replace the destroyed cells. Low red blood cell count Low hemoglobin levels These signs and symptoms usually have a relatively sudden onset, appearing over a few days.

Possible Causes

In approximately half of all autoimmune hemolytic anemia cases, the condition is classified as “idiopathic”, meaning the cause is unknown. Alternatively, it may be caused by medications or other underlying disease. The cause of the signs and symptoms of autoimmune hemolytic anemia are directly related to the destruction of red blood cells in the blood. These low levels are a result of circulating antibodies that bind to the blood cells and destroy them. Antibodies are made under normal conditions against foreign substances in the body and are therefore very useful in warding off infection. In conditions that are referred to as “autoimmune” the body makes antibodies against itself. The severity of the condition is related to how many antibodies, and which kinds specifically, are attached to each RBC. In the case of autoimmune hemolytic anemia, it is not currently known what triggers the formation of the antibodies.

Diagnosis

In diagnosing autoimmune hemolytic anemia, it will first be important for your child’s physician to rule out other underlying diseases that may be causing the condition. Examples of such diseases are systemic lupus erythematosus, non-Hodgkin lymphoma, Hodgkin disease, cancer, HIV and chronic ulcerative colitis. Appropriate tests to rule out each of these diseases may be ordered depending on which specific signs and symptoms the patient presents with. These disorders are unusual in children with autoimmune hemolytic anemia though. Upon ruling out other causes of the anemia, the presentation of the patient with the specific physical findings previously described, as well as the associated laboratory findings, may lead to a diagnosis of autoimmune hemolytic anemia.

Treatment

The treatment for idiopathic cases of the condition involves stopping the destruction of the RBCs and replacing them if necessary. Transfusions of blood are often necessary to keep the anemia from becoming too severe. These transfusions are only temporizing measures though, because the transfused RBCs get destroyed as well. For this reason, the child may be given a corticosteroid such as prednisone which reduces the destruction of the RBCs. Further treatment may be necessary with immune system suppressing drugs to block the production of the antibodies. In more severe cases that are unresponsive to treatment, it may become necessary to remove the spleen, or use IVIG (immunoglobulin) therapy, or both. The spleen is the organ that is responsible for removing the platelets and RBCs from circulation in the blood once they have been marked with the antibodies. A person can live a normal life without the spleen but special measures need to be taken, such as vaccinations and daily penicillin treatment, to prevent dangerous infections.

Prognosis

The prognosis of a child with autoimmune hemolytic anemia depends greatly on the child’s response to treatment. Most children have diseases that eventually resolve completely on their own, over about 6 months. Adults who acquire the condition usually have several reoccurrences during a lifetime. During periods of time when the person is anemic, he/she may be at risk for reversible heart failure. The low levels of RBC put extra strain on the heart as it works to compensate. Watching for development of signs of complications of anemia will be crucial in determining the patient’s prognosis. If the person responds well to treatment and the episode of anemia is short-lived, he/she can expect to live a normal life. Those with chronic disease, or who have other underlying autoimmune problems, may require therapy for an extended period of time, perhaps life-long.

Connect with other parents

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Weblinks

Medline Plus
This link is to a webpage of the National Library of Medicine through the NIH. The page offers more easy to read information on the condition.

American Autoimmune Related Diseases Foundation
A website to get information on this disease. You must request the information to be sent to you, but you only pay for postage.

University of Pennsylvania Health System
A nice, easy to read review of the disease with definitions of many of the medical terms.

Google Search for Autoimmune Hemolytic Anemia

References and Sources

www.nlm.nih.gov/medlineplus/ency/article/000579.htm www.aarda.org www.rarediseases.org