Overview

The hemolytic anemias are a group of disorders in which the red blood cells are destroyed faster than the bone marrow can make them. The term for destruction of red blood cells is hemolysis.

Causes

Hemolytic anemia may have intrinsic or extrinsic causes.

  • Intrinsic. This is when the destruction of red blood cells is due to a flaw within the red blood cells. This type is often inherited. It includes sickle cell anemia, thalassemia, or erythrocytosis. These conditions produce red blood cells that don't live as long as normal red blood cells.
  • Extrinsic. This is when the destruction of red blood cells is from a cause outside of the cells. Some of the causes of extrinsic hemolytic anemia, also called autoimmune hemolytic anemia, are:
    • Infections. From bacteria or viruses.
    • Medicines. Antibiotics like penicillin or sulfonamides, medicines for malaria, or acetaminophen.
    • Cancers. Leukemia, lymphoma, or other tumors.
    • Autoimmune disorders. Systemic lupus erythematous (SLE or lupus), rheumatoid arthritis, Wiskott-Aldrich syndrome, or ulcerative colitis.
    • Hypersplenism. A condition in which the spleen destroys more red blood cells than normal.

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

Symptoms

These are the most common symptoms of hemolytic anemia:

  • Pale 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
  • Fast heart beat
  • Heart murmur

The symptoms of hemolytic anemia may look like other health problems. Always check with your child's doctor for a diagnosis.

Diagnosis

Your child’s healthcare provider may suspect hemolytic anemia from child's medical history and a physical exam. Your child may have these tests:

  • Hemoglobin and hematocrit. It measures the amount of hemoglobin and red blood cells in the blood.
  • Complete blood count, or CBC. This test checks the red and white blood cells, blood clotting cells (platelets), and sometimes, young red blood cells (reticulocytes). It includes hemoglobin and hematocrit and more details about the red blood cells.
  • Peripheral smear. A small sample of blood is viewed under a microscope. Blood cells are checked to see if they look normal or not.
  • Other blood tests. These tests will check lactate dehydrogenase, bilirubin, haptoglobin, and certain antibodies.
  • Urine tests. For example, to check for hemoglobin from red blood cells breaking down.
  • Bone marrow aspiration, biopsy, or both. A procedure in which a small amount of bone marrow fluid (aspiration), solid bone marrow tissue (called a core biopsy) often from the hip bones, or both are checked for the number, size, and maturity of blood cells. This test may also look for abnormal cells.

Treatment

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.

A child with hemolytic anemia is often treated by a hematologist, an expert in blood disorders. The treatment for hemolytic anemia will vary depending on the cause of the illness and how severe the anemia is. Some children do not need treatment. For those that do, treatment may include:

  • Blood transfusions
  • Corticosteroid or steroid medicines
  • Intravenous immune globulin (IVIg)
  • Rituximab, a medicine to control the immune system
  • Antibiotics to treat the infection if that is the underlying cause
  • Other medicines, depending on the type of hemolytic anemia

In some children, who don't respond to first-line therapy, these treatments may be needed:

  • Splenectomy. Surgical removal of the spleen.
  • Immunosuppressive therapy. Use of other drugs to control the immune system.
  • Plasmapheresis. Removal of antibodies from the blood that are destroying red blood cells.
  • Stem cell transplant. Damaged bone marrow cells are replaced with donor bone marrow.

Complications

Complications depend on the cause of the hemolytic anemia and include:

  • Side effects of medicines (for example, the increased risk of infection with corticosteroids)
  • Gallstones or stones in the gallbladder
  • Blood clots and pulmonary emboli (blood clots in the lungs)
  • Kidney failure
  • Shock
  • Heart failure

Living with

Managing hemolytic anemia depends on how severe the symptoms are. Children with mild symptoms will have regular blood checks.

Managing children with more severe symptoms is more involved. These children will also have their blood checked regularly. They may have trouble with daily activities due to their symptoms or treatment. They should also avoid people who are sick, if taking medicine that increases the chance of infection.

When to Call a Healthcare Provider

Call your child's healthcare provider if your child's behavior changes. For example, your child may not be able to be as active as usual. Also call your child's provider if your child has yellowing of the skin, dark urine, or a fever.

Key Points

  • Hemolytic anemias are a group of conditions in which red blood cells are destroyed.
  • Hemolytic anemia may be an inherited condition or it may be from autoimmune conditions, infections, cancers, or medicines.
  • Many children do not need treatment. If they do, common treatments are blood transfusions, steroids, and other medicines.

Next Steps

Tips to help you get the most from a visit to your child’s healthcare provider:

  • Know the reason for the visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
  • Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
  • Ask if your child’s condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if your child does not take the medicine or have the test or procedure.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.