Hemolytic Anemia in Children

What is hemolytic anemia?

Hemolytic anemia is a disorder in which the red blood cells are destroyed faster than the bone marrow can produce them. The term for destruction of red blood cells is "hemolysis." There are two types of hemolytic anemia:

• Intrinsic. The destruction of the red blood cells is due to a defect within the red blood cells themselves. Intrinsic hemolytic anemias are often inherited, such as sickle cell anemia, thalassemia, or erythrocytosis. These conditions produce red blood cells that do not live as long as normal red blood cells.

• Extrinsic. Red blood cells are produced healthy but are later destroyed by getting trapped in the spleen, destroyed by infection, or destroyed from drugs that can affect red blood cells. In severe cases the destruction takes place in the circulation. The following lists some of the causes of extrinsic hemolytic anemia, also called autoimmune hemolytic anemia:

  • Infections (hepatitis, cytomegalovirus (CMV), Epstein-Barr virus (EBV), typhoid fever, E. coli, or streptococcus)

  • Medications (such as penicillin, antimalaria medications, sulfa medications, or acetaminophen)

  • Leukemia or lymphoma

  • Autoimmune disorders (such as systemic lupus erythematous (SLE, or lupus), rheumatoid arthritis, Wiskott-Aldrich syndrome, or ulcerative colitis)

  • Various tumors

  • Hypersplenism

Some types of extrinsic hemolytic anemia are temporary and resolve over several months. Other types can become chronic with periods of remissions and recurrence.

What are the symptoms of hemolytic anemia?

The following are the most common symptoms of hemolytic anemia. However, each child may experience symptoms differently. Symptoms may include:

• Abnormal paleness or lack of color of the skin

• Jaundice, or yellowing of the skin and eyes

• Dark colored urine

• Fever

• Weakness

• Dizziness

• Confusion

• Intolerance to physical activity

• Enlargement of the spleen and liver

• Increased heart rate (tachycardia)

• Heart murmur

The symptoms of hemolytic anemia may resemble other blood conditions or medical problems. Always consult your child's physician for a diagnosis.

How is hemolytic anemia diagnosed?

Hemolytic anemia may be suspected from general findings on a complete medical history and physical examination of your child, such as complaints of tiring easily, pale skin and lips, or a fast heartbeat (tachycardia). In addition to a complete physical examination, your child's physician may order the following diagnostic tests:

• Blood tests. These tests measure hemoglobin and reticulocyte count and will reveal how many new red blood cells are being produced.

• Additional blood tests. These tests will check liver function as well as the presence of certain antibodies.

• Urine tests for hemoglobin.

• Bone marrow aspiration and/or biopsy. A procedure that involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow tissue (called a core biopsy), usually from the hip bones, to be examined for the number, size, and maturity of blood cells and/or abnormal cells.

Treatment for hemolytic anemia

Specific treatment for hemolytic anemia will be determined by your child's physician based on:

• Your child's age, overall health, and medical history

• Extent of the anemia

• Cause of the anemia

• Your child's tolerance for specific medications, procedures, or therapies

• Expectations for the course of the anemia

• Your opinion or preference

The treatment for hemolytic anemia will vary depending on the cause of the illness. Treatment may include:

• Blood transfusions

• Corticosteroid medications

• Treatment with intravenous immune globulin (to strengthen the immune system)

• Rituximab

In more severe cases, the following treatments may be necessary and usually require hospitalization:

• Exchange transfusion (similar to a blood transfusion but with more blood being given and an equal amount of the child's hemolyzed blood being removed)

• Surgical removal of the spleen (usually reserved for children who do not respond to other therapies)

• Immunosuppressive therapy