When the body fights its own red blood cells
Autoimmune Hemolytic Anemia (AIHA) is a blood disease in which a person produces substances that cause their own body to destroy red blood cells (RBCs), resulting in anemia (low hemoglobin).
To help us understand this process, here is a little background information on the key players:
All blood cells are made in the bone marrow. This is the red, spongy part inside the bones. There are three basic types of blood cells: white blood cells which fight infection, platelets that stop bleeding, and red blood cells which carry oxygen throughout the body in the form of hemoglobin. If the hemoglobin is low for any reason, the result is anemia.
Antibodies are substances produced by white blood cells. They are like sticky little sweaters which coat germs or anything else that shouldn’t be in your body. A cell coated with antibodies is also like carrying a little neon sign that tell other white blood cells “Here’s the enemy, now get it!”
In AIHA, the red blood cells are produced normally in the bone marrow. The problem arises when the body produces an antibody that coats the red blood cells.
When the red blood cells wearing the “antibody sweaters” circulate through the body, they are recognized as “enemy” cells and destroyed. The two major blood filtering organs in the body are the liver and spleen. These are the places the antibody coated cells are recognized as foreign and destroyed. When the antibody is destroyed, so is the red blood cell. This results in anemia.
The body will try to make up for this loss by increasing the red blood cell production in the bone marrow. One of the ways to see this effect is by measuring the amount of immature, young, red blood cells in the circulating blood. These are called “reticulocytes”. The reticulocyte count tells us how hard the body is working to make new red blood cells to replace the ones recently destroyed.
One way to determine if the body is producing antibodies directed against itself is with a blood test called a Coombs. The Coombs test is often positive in children with AIHA.
How did this antibody come to be?
Why the body produces an antibody directed at its own red blood cells is unknown in most cases.
It can be associated with certain underlying diseases like rheumatoid arthritis, lupus, ulcerative colitis, thyroid disease, chronic active hepatitis, or certain immunodeficiency syndromes. The anemia can be a long-standing problem for many.
Antibody production against red blood cells is also associated with certain infections, most commonly, respiratory viruses. A bacterial infection called Mycoplasma Pneumonia is also associated with development of AIHA. The antibodies and anemia tend to go away after the infections are completely resolved.
It is possible to inherit antibodies from a mother at birth. This is usually temporary.
Will it go away?
In most cases, AIHA comes on acutely, or quickly. Depending on what triggered the antibody production, it can resolve within a few months or last years.
In about half of children diagnosed over the age of ten, AIHA can become a chronic problem.
What treatments are available?
It is common that children with AIHA require red blood cell transfusion support. This corrects the severe anemia which could be life threatening. A blood transfusion does not correct the underlying cause of the AIHA, but it prevents serious complications from severe anemia.
Treatment for AIHA is directed at getting rid of the antibody. This can be done a couple of different ways. There are oral medications that can be given to suppress the body’s ability to make the antibody. This acts to “quiet” the person’s overactive immune system.
Another approach is to use intravenous medications to “trick” the spleen into ignoring the antibody coated red blood cells as they circulate.
Another way to get rid of the antibody is to remove it with an intravenous treatment called pheresis (for-e-sis). This involves washing the blood through a machine that has an “antibody magnet” to attract and destroy the antibodies, then return the normal cells back to the body.
What should I look for?
The signs of anemia are paleness, tiredness, headache, shortness of breath, dizziness, or heart palpitations.
Other things to look for are yellow tint in the skin or whites of the eyes, and dark urine (the color of tea or coke). These are signs that the body is destroying red blood cells, and anemia will result.
If you see any of these, call your doctor promptly.
How can I help my child deal with this?
The diagnosis of AIHA means that your child’s activities and routines will be disrupted. Your child may feel tired, cranky, angry, and afraid. You and your child are suddenly faced with frequent blood tests, medications, and doctor visits. Truthful, honest communication with your child ensures trust and is usually the best approach. Assure the child that nothing they did caused this problem. Make them feel secure knowing that you and the doctors will be giving them special care to help them get well. Be honest about doctor visits, blood tests and medications needed. Younger children often handle these situations better with shorter notice (the day or morning of a blood test, for example), while older children may want time to prepare themselves. Create a reward system for their cooperation and tolerance of unpleasant tasks associated with having AIHA.
Encourage your child to listen to their body cues. They should rest when tired, and report symptoms of anemia. Assure them that they will not fail at school or other activities if they need to slow down for rest.
Be creative with activities that can be done without physical exertion. Promote continued school work as tolerated. If your child is unable to attend full days, get homework in advance or make arrangements with the school for the child to do self- paced work.
Is there anything else I can do?
Promoting a well balanced diet, plenty of fluids and rest are important. Your child should try to eat something from all four food groups in several small feedings each day. Use of any dietary supplements, vitamins, or herbals should be discussed with your doctor before use, since some may interfere with the treatment.
The IHTC clinical team works in close partnership with parents. You are the person who knows your child best. Our medical team will assess the information and observations you report along with other physical monitoring to provide your child with the best care.
How the IHTC can help you
The IHTC is committed to providing expert care for infants, children, teens, and adults with hemochromatosis. As a center of excellence in the treatment of bleeding and clotting disorders, the IHTC offers a high level of coordinated care for patients with this disorder. We also provide prompt communication with your local healthcare provider related to our treatment plan and your progress. The IHTC team collaborates with national experts and patient participation in research studies may be available.
At the IHTC we have clinicians who are experts in treating hemochromatosis and providing support to patients with this disorder. As every patient can experience a different rate of disease progression and different symptoms it is very important that you are seen by a clinician who is familiar with the signs and treatment of hemochromatosis. We can help you with diagnosis, testing and treatment; and can provide dietary, genetic and general counseling depending on your specific needs. For more information, please contact us at 877.CLOTTER (877.256.8837).