Autoimmune hemolytic anemia (AIHA) is a rare immune condition that occurs when your body attacks and destroys healthy red blood cells (hemolysis). The destruction of the red blood cells by antibodies causes anemia and can lead to a range of health problems. There are multiple treatment approaches for people living with AIHA. Generally, AIHA can be well managed, but in some cases it is fatal if not treated.

What Medications Can Manage AIHA?

If your AIHA symptoms are mild, treatment may not be needed, and your health care provider may closely monitor your red blood cell levels. Medications are often prescribed for people with AIHA when symptoms are more severe or if the red blood cells are being destroyed rapidly.

The best treatment for you depends on your type of AIHA. There are two main types of AIHA — cold AIHA and warm AIHA. The warm and cold classification depends on the type of antibodies that are involved.

Many of the U.S. Food and Drug Administration (FDA)-approved medications used to treat AIHA are prescribed off-label. This means that the drug was not specifically approved for use in people living with AIHA. Because AIHA is a rare condition, it may be challenging for researchers to meet certain criteria for full FDA approval of drugs that benefit people with AIHA. Ask your health care provider if you have concerns about using an off-label medication to treat your AIHA.

Targeted Therapies

Targeted therapies are medications that only block or affect specific cells or molecules in the body. Because these drugs only target certain cells, they may cause fewer side effects.

Rituximab (Rituxan) is the first-line therapy for warm and cold AIHA. The medication targets the white blood cells (B cells) that create the autoantibodies responsible for destroying the red blood cells. Rituximab may be prescribed by itself or given with other chemotherapy or immunosuppressive medications.

Sutimlimab-jome (Enjaymo) was recently approved specifically for people living with cold agglutinin disease, a type of cold AIHA. This drug is a monoclonal antibody that blocks proteins that activate the harmful autoimmune B cells.

Corticosteroids

Corticosteroids are a type of anti-inflammatory drug often used to treat autoimmune disorders.

This medication is commonly prescribed to people living with warm AIHA, but its effectiveness is less clear in people with cold AIHA. Corticosteroids are often started at a high dose and then slowly reduced over a period of weeks or months. The medication can be given with other therapies such as rituximab. Corticosteroids used to treat warm AIHA include prednisone, prednisolone, methylprednisolone, and dexamethasone.

Chemotherapy and Immunosuppressive Medications

Chemotherapy medications may be prescribed to treat warm or cold AIHA. You may generally think of chemotherapy medications only being used to treat cancer. Medications used to treat blood cell cancer can be effective with AIHA because they may reduce the number of immune cells that produce the autoantibodies that attack the body’s red blood cells.

The following immunosuppressive medications may be prescribed to reduce the activity of the immune cells that produce harmful autoantibodies:

• Mycophenolate mofetil — This immunosuppressive therapy may be combined with rituximab in people with warm AIHA, but it is not as effective in people with cold AIHA.
• Cyclosporine — This immunosuppressive medication is generally reserved for children with refractory AIHA (disease that has not responded or improved despite past treatments).
• Cyclophosphamide — This drug is an immunosuppressive chemotherapy medication that is often prescribed to people with severe refractory warm AIHA.
• Azathioprine, tacrolimus, and sirolimus — These immunosuppressive drugs are not commonly prescribed unless a person has severe refractory AIHA.

You may experience side effects from chemotherapy and immunosuppressive drugs, including tiredness, nausea, vomiting, and a higher risk of infection. Talk to your doctor about any concerns you have regarding your medications or treatment plan.

Erythropoiesis-Stimulating Agents

Erythropoiesis-stimulating agents are drugs used to treat anemia in people with chronic kidney disease or who are undergoing chemotherapy. Erythropoiesis-stimulating agents are also sometimes used off-label to treat AIHA in people who haven’t responded to other treatments. These drugs help the body produce more red blood cells.

Treatment for Underlying Conditions

When another condition contributes to the development of AIHA, it is called secondary AIHA. AIHA that is not associated with another health condition is called primary or idiopathic AIHA. One of the best approaches to treating secondary AIHA is to treat the condition that is causing the anemia. Secondary AIHA may be triggered by the following diseases or conditions:

• Infections — Viruses, including Epstein-Barr virus, measles, mumps, rubella, cytomegalovirus, hepatitis, HIV, and varicella (the virus that causes chickenpox and shingles)
• Cancer — Lymphoma, leukemia, colon cancer, and other solid tumors
• Autoimmune diseases — Systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome, thyroid disease, ulcerative colitis, Hashimoto’s disease
• Drug-induced hemolysis — Drug-induced immune hemolytic anemia, a rare condition caused by some medications

If you have a condition that has been linked to AIHA, treating that condition should help reduce the destruction of red blood cells and thus reduce your AIHA symptoms.

What Medical Procedures Can Help Manage AIHA?

Sometimes surgery or other medical procedures are needed to complement medications or provide faster results. If you have severe AIHA symptoms or are not responding to medications, your health care provider may prescribe the following procedures:

• Blood transfusion — This procedure offers temporary relief for people with severe anemia by providing new red blood cells.
• Plasmapheresis (plasma exchange) — This procedure temporarily reduces the amount of red blood cell-destroying antibodies in people with AIHA.
• Intravenous immunoglobulin — This procedure involves administering antibodies (immunoglobulin) in the vein.
• Splenectomy — Removing the spleen is a procedure that is effective for some individuals with warm AIHA when other treatments have failed. It is not an effective therapy for those with cold AIHA.
• Stem cell transplantation — This treatment is only used in people with AIHA if they are not responding to any of the medications or other procedures.

Ongoing Research in AIHA Treatments

Clinical trials are studies that help researchers learn more about how new or existing drugs can treat AIHA. All drugs must pass through clinical trials to test their safety and efficacy to gain approval from the FDA.

Several clinical trial options are available for people with warm or cold AIHA. Talk with your health care provider to learn more about whether you are eligible to participate in a clinical trial.

What Lifestyle Changes Can Help Manage AIHA?

Certain lifestyle changes can help manage your AIHA. If you are living with cold AIHA, it is important to avoid the cold. If you must be in cold temperatures, protecting the head, face, hands, and feet can help decrease the red blood cell hemolysis. In case of a medical procedure or hospitalization, solutions given through an IV should be warmed for people with cold AIHA.

Condition Guide

• What Are the Common Symptoms in People With AIHA?
• What Causes AIHA?
• What Are the Different Types of AIHA?
• How Is AIHA Diagnosed?
• What Is AIHA? An Overview of Autoimmune Hemolytic Anemia
• Conditions Related to AIHA: What You Should Know