Autoimmune Hemolytic Anemia (AIHA)

What is autoimmune hemolytic anemia?

Symptoms of AIHA

Signs and symptoms of AIHA include:

• Fatigue or feeling tired
• Pale skin, gums, or nail beds
• Headache 
• Dizziness or feeling lightheaded
• Cold hands or feet
• Fast or irregular heart rate
• Shortness of breath     
• Yellow color of skin and eyes (jaundice)
• Dark urine

Causes of AIHA

AIHA is a disorder of the immune system. In children with AIHA, the body makes red blood cells normally, but the immune system attacks and destroys them by mistake. The breakdown of red blood cells is called hemolysis.

The immune system's job is to attack germs that should not be in the body. In an autoimmune condition like AIHA, the body’s immune attacks the body’s own healthy cells. 

In about half of the AIHA cases, the cause of autoimmune hemolytic anemia is unknown. This is called primary AIHA.

In other cases, AIHA is linked to another cause or underlying condition. This is called secondary AIHA. Factors associated with secondary AIHA include some autoimmune diseases, cancers, infections, medications, and stem cell (bone marrow) transplant.

Diagnosis of AIHA

Your child’s doctor may refer your child to a hematologist, a doctor who diagnoses and treats blood disorders. AIHA is diagnosed based on your child’s medical history, physical exam, and lab tests. 

Lab tests may include:

A complete blood count (CBC) to look for signs of anemia. A CBC test measures:

• The number of red blood cells, white blood cells, and platelets  
• The size of red blood cells
• Hematocrit, which shows how much of the blood is made up of red blood cells 
• Hemoglobin, a protein in your blood that carries oxygen 

Other tests may include:

• Peripheral blood smear to look at blood cells under a microscope. 
• Reticulocyte count to measure the number of young red blood cells in your child’s body. A high reticulocyte count means the bone marrow has to make more cells to replace damaged ones.
• Bilirubin test to measure bilirubin levels. Bilirubin is a substance made by the liver when red blood cells are broken down.  
• Direct antiglobulin test (Coombs test) to see if the body is making antibodies against red blood cells.
• Haptoglobin test to measure haptoglobin, a protein that gets rid of substances made by damaged red blood cells. Your child will have low levels if their body is using haptoglobin for this purpose.

Types of AIHA    

There are different types of AIHA but they fall into 2 main categories: warm and cold.

Warm autoimmune hemolytic anemia

Warm AIHA is the most common form of primary AIHA in children. It makes up 60–90% of cases. It is called warm AIHA because antibodies attack red blood cells when blood is at normal body temperature or higher-than-normal body temperature.

Cold autoimmune hemolytic anemia

Cold AIHA is rare in children, making up about 10% of cases. If your provider suspects cold AIHA, they may order tests to measure antibodies that attack red blood cells at cold temperatures.

Types of cold disease include:

• Cold agglutinin disease (CAD): CAD usually happens after infection with Epstein-Barr virus or a type of bacteria called Mycoplasma pneumoniae. It happens when the immune system attacks red blood cells when blood is at a lower-than-normal body temperature. 
• Paroxysmal cold hemoglobinuria (PCH or Donath-Landsteiner syndrome): PCH is a rare type of cold disease but it is more common in young children. Underlying causes of PCH include viral infections, syphilis, and other bacterial infections. Red blood cell damage results from exposure to cold. In children, this disease often gets better on its own once exposure to cold stops.

Treatment of AIHA

Treatment for AIHA is based on the type (warm or cold) and severity of anemia. Not all children need treatment for AIHA. Many children recover on their own. Your care team may recommend monitoring or watchful waiting. The hematology team will check your child’s blood counts and symptoms to see when and if treatment is needed. 

If your child’s AIHA is linked to another condition, it is important to identify and treat the underlying cause.

Corticosteroid medicines

The first choice of treatment for primary AIHA is a corticosteroid (steroid) medicine, such as prednisone. Your child will take the medicines by mouth. 

Steroids can have different side effects. These may include:

• Stomach pain
• Feeling more hungry than usual
• Moodiness
• Trouble sleeping
• Flushing of the face
• Weight gain, especially in the face and belly
• Acne
• High blood pressure
• High blood sugar level
• Increased risk of infection

Your child’s doctor may prescribe other medicines to help your child with side effects. Your child’s doctor will gradually decrease the dose of the steroids until they can be stopped completely. The side effects go away after your child stops taking the medicine.

Immunosuppressant medicines

In some cases, steroids do not work well. Treatments may include other medicines to suppress the immune system (immunosuppressants). These may include rituximab, intravenous immune globulin (IVIG), and other medicines.  

Blood transfusion

In severe cases, children may require a blood transfusion.

Splenectomy

If other treatments do not work, your doctor may recommend surgery to remove the spleen (splenectomy). The spleen filters the blood and removes abnormal red blood cells from the body. Removing the spleen can help increase the number of red blood cells to treat the anemia.

Treatments for cold AIHA

In many cases of cold agglutinin disease or PCH, anemia symptoms are mild and go away on their own. It is important to treat the infection or other underlying cause. 

Mild cold AIHA can be treated with rest and by avoiding cold. Take steps to keep your child warm by staying out of cold environments, increasing room temperatures, wearing warm clothing, using blankets and heaters, and avoiding cold foods and beverages. Your care team will help you know what steps to take and how long your child needs to avoid cold temperatures.     

Prognosis for AIHA

The course of AIHA varies, depending on the type and the response to treatment. Most children only have AIHA for a few weeks to months. Some children will have AIHA that comes and goes over months to years and may need ongoing therapy. There is no way to know how quickly your child will recover from AIHA.

About 90% of children with AIHA will achieve remission. But the condition can be life-threatening if not treated. It is important to seek immediate medical care. Your child’s care provider will talk with you about your child’s diagnosis, treatment options, and monitoring needed.

When to call your care team

Contact your child’s care team if your child:

• Looks pale
• Has increased yellowing of the skin
• Has dark urine
• Is more tired than usual

Talk to your care team about any questions or concerns you may have.

Questions to ask your care team

• What type of AIHA does my child have?
• What are my child’s treatment options?
• How will AIHA affect my child’s daily life?
• What symptoms do I need to watch for and report immediately?
• What is the long-term outlook for my child?
• What follow-up appointments and medical care will my child need?

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