Autoimmune Hemolytic Anemia: Symptoms, Causes, Treatment

Written by Hallie Levine
Medically Reviewed by Laura J. Martin, MD on May 27, 2025
12 min read

Autoimmune hemolytic anemia (AIHA) is a rare type of anemia that is diagnosed in only about 1-3 people per 100,000.  

Anemia is when your bone marrow doesn't make enough red blood cells. Or the red blood cells you do have don't work well.

Red blood cells carry oxygen throughout your body. They’re made in the spongy material deep inside your bones called bone marrow. These cells normally live for about 120 days. When you have too few red blood cells, your body can't get enough oxygen, which leaves you feeling tired or short of breath.

If you have AIHA, your body’s immune system attacks and destroys red blood cells faster than your bone marrow can make them. Sometimes your red blood cells live for just a few days.

AIHA is divided into two types:

Primary AIHA. It’s not caused by another condition.
Secondary AIHA. It’s caused by another condition, such as viral illness or other autoimmune issues.  

The temperature at which damage occurs also determines the type, warm or cold:

Warm autoimmune hemolytic anemia. This immune reaction takes place at or above normal body temperature. It’s the most common type of AIHA. Your body makes specific antibodies known as IgG. These antibodies destroy red blood cells faster than your body can make them.

Cold autoimmune hemolytic anemia. Also known as cold agglutinin disease (CAD), this is when your body makes specific antibodies known as IgM. These antibodies destroy red blood cells when you’re exposed to cold, usually at about 37 to 39 degrees Fahrenheit. It’s extremely rare. About 1 person per 1 million develops it each year, usually between the ages of 40 and 80.

Many of the symptoms of AIHA are the same as those of other forms of anemia. They include:

  • Weakness and fatigue
  • Chills
  • Fast heart rate, known as tachycardia
  • Pale skin
  • Shortness of breath
  • Chest pain
  • Yellow skin or whites of the eyes turn yellow (jaundice)
  • Dark pee
  • Feeling like your belly is full, which is related to an enlarged spleen

What is the first sign of autoimmune hemolytic anemia? 

Some people don’t have any symptoms, especially if red blood cell damage is mild or happens over time. Others have typical anemia symptoms like fatigue and weakness.

Other symptoms of autoimmune hemolytic anemia

With cold antibody hemolytic anemia, you may also notice:

  • Cold hands and feet
  • Bluish or reddish color in hands and feet
  • Headache
  • Digestive problems
  • Pain in the back and legs

Other autoimmune diseases, like lupus, can lead to AIHA. Other conditions and medications that may trigger it include:

Is autoimmune hemolytic anemia contagious?

No. It’s an immune disorder, so you can’t pass it to others or get it from someone else. But the viruses that can lead to AIHA, like Epstein-Barr, are contagious. They spread through bodily fluids like semen, blood, and saliva.

You’re more at risk for AIHA if:

  • You have another autoimmune disease, like lupus.
  • You have certain cancers, such as chronic lymphocytic leukemia.
  • You take medications like methyldopa (Aldomet), penicillin, quinine (Qualaquin), and sulfonamides.
  • You have or have had a virus like HIV, Epstein-Barr, or cytomegalovirus.

You’re also more likely to develop CAD if:

  • You’re between the ages of 40 and 80.
  • You’re female.
  • You live in a colder climate.

Talk to your doctor if you think you might have anemia of any type. They’ll ask about your symptoms, any medications you take, and other important information. They may refer you to a hematologist, a doctor who specializes in blood diseases. 

Tests for autoimmune hemolytic anemia 

The first test your doctor will order is a complete blood count, or CBC. This test measures:

  • The number of red blood cells, white blood cells, and platelets
  • The size of your red blood cells
  • The protein in your red blood cells that carries oxygen, called hemoglobin
  • How much space red blood cells take up in your blood, or hematocrit

A low red blood cell count, along with low hemoglobin and hematocrit levels, are signs of anemia.

If CBC test results point to anemia, your doctor might want to do more tests, such as: 

Reticulocyte count. This measures the number of young red blood cells in your body. A high reticulocyte count means your bone marrow is making a lot more cells to replace the ones your body has destroyed.

Coombs' test. This shows if your body is making antibodies against red blood cells.

Peripheral smear. Your doctor will check your red blood cells under a microscope for signs of blood cell destruction.

Bilirubin test. Levels of this substance go up when blood cells are destroyed.

Haptoglobin test. This protein clears debris from damaged red cells out of the bloodstream. Levels will be low when it’s being used heavily.

Cold agglutinin titer. This measures the amount of the antibodies that attack red blood cells at cold temperatures.   

What to know when you’re diagnosed

It’s important to find out if an underlying condition is causing your AIHA. Ask your doctor what additional tests may help pinpoint the cause. 

How to deal with a diagnosis

It’s understandable to feel overwhelmed or anxious about the future when you’ve been diagnosed with a rare condition. Tips to help you manage these feelings include:

Educate yourself. The more you know, the more empowered you are. Write down a list of questions you’d like answered and take them to your next appointment. Your doctor can also point you to reputable sources of information online. 

Talk to someone. Sharing your feelings with a trusted friend or family member can lighten the load. But it’s best to reach out to a licensed mental health professional if symptoms of depression show up, such as persistent sadness or a loss of interest in activities you once enjoyed. If cost is a concern, look for therapists who offer services based on your income, also known as a sliding scale.

Take care of yourself. Try your best to eat healthy foods, exercise, and make time for activities that bring you joy. 

Focus on what you can do. For instance, if you’re an outdoorsy person who loves to hike, you might not be able to complete a hilly and strenuous 10-mile trail because of fatigue and weakness. But you may be able to complete 3 miles on a moderate trail. 

Set up a support system. These are the people who step in when you’re too fatigued or weak to go grocery shopping, do laundry, and other day-to-day tasks.  

Your medical team may include these health care professionals:

Primary care doctor. They provide routine day-to-day care and make sure you’re up-to-date on all necessary screening tests and vaccinations.

Hematologist. This doctor specializes in blood disorders. Many hematologists also specialize in treating blood cancers. They’re called hematologist oncologists. 

Immunologist. These doctors specialize in diseases of the immune system and can help you manage AIHA and any other autoimmune conditions you may have.

Nutritionist. They’ll work with you to create a balanced diet and discuss if you need folic acid supplements.

Cardiologist. The risk of forming blood clots is higher when you have AIHA. A heart doctor may want to monitor you for any other risk factors.

 

You may not need treatment if your AIHA is mild. If an existing condition triggered AIHA, your doctor will treat that first. If a medication is the cause, you'll likely have to stop taking it. Your doctor will let you know if there’s another medication you can replace it with.

Medications for autoimmune hemolytic anemia

Steroids. When treating warm autoimmune hemolytic anemia, doctors usually prescribe a steroid first, such as prednisone, to stop your immune system from attacking red blood cells.

Rituximab. This synthetic antibody that targets the substances that put your immune system in overdrive.

Enjaymo (sutimlimab-jome). The FDA approved this drug in 2022 to treat cold autoimmune hemolytic anemia. It helps stop your body from destroying red blood cells.

Immunosuppressants. Other medicines, such as azathioprine (Imuran) and cyclophosphamide FDA approves treatment for adults with rare type of anemia can be used to curb your immune system response.

Surgery for autoimmune hemolytic anemia

Surgery to remove your spleen is also an option in the treatment of warm autoimmune hemolytic anemia. Much of the destruction of red blood cells takes place in the spleen. Surgery is often considered if medications don't help or if you can’t tolerate them.

You may need a blood transfusion if you have a severe case.

Natural treatments for autoimmune hemolytic anemia

Your doctor may want you to take a folic acid (B9) supplement. People with AIHA are often low in this vitamin, which helps your cells work properly. The usual recommended dose is 1 milligram per day.

They may also recommend a vitamin D supplement because it can be hard to get it from foods alone. Be sure to tell your doctor everything you’re taking, even if it’s not prescription medication. Some supplements can interfere with prescription medications. They can tell you what’s safe and what isn’t.

Home remedies for autoimmune hemolytic anemia

If you have CAD, you’ll want to avoid the cold, which can worsen symptoms. Protect yourself with these tips:

  • Wear gloves whenever you take food out of your refrigerator or freezer.
  • Wear a hat, scarf, coat, and gloves whenever you’re outdoors in cold weather.
  • Don’t blast the air at home.
  • Wear layers to stay warm if you know you’re going somewhere with very cold air conditioning.
  • Warm up your car before you get in when it’s cold out.

AIHA is unique among rare diseases because it doesn’t have specific stages. Symptoms can come on gradually or appear suddenly, triggering life-threatening anemia.

If left untreated, AIHA can lead to potentially life-threatening complications. These include:

Skin problems

People with CAD often have less red blood cell flow to their hands and feet, especially when they’re cold or in a cold environment. This may cause skin ulcers and skin discoloration. Sometimes, skin tissue may die. 

Blood clots

Up to a quarter of people with AIHA form blood clots in their legs. They can also form pulmonary emboli, or blood clots in their lungs.

Kidney failure

AIHA can damage your kidneys if it’s not well controlled.

Life with AIHA can be challenging, but there are steps you can take to make it easier. It’s especially important to focus on a healthy diet, exercise, and your overall mental health.

Autoimmune hemolytic anemia diet

A well-balanced diet can help promote your body’s production of healthy red blood cells. Here are some nutrients to make sure you have in your diet:

Iron-rich foods. Your body needs iron to make hemoglobin, the protein in your red blood cells that carries oxygen. Good food sources include lean meats, poultry, fish, legumes, and dark leafy greens. Eat them alongside foods rich in vitamin C, like citrus fruits, to boost iron absorption.

Vitamin B12. Low levels of this nutrient can worsen anemia. You can get it through dairy products, eggs, fish, and fortified cereals.

Vitamin B9 (folate). This vitamin helps your body make red blood cells and encourages them to grow. Good sources include green leafy veggies, legumes, fortified cereals, and citrus fruits.

Vitamin D. Low levels have been linked to autoimmune conditions. It also helps regulate your immune system. Look for it in fatty fish and fortified dairy products. 

Antioxidant-rich foods. They can help ease the inflammation that comes with autoimmune disorders. Good sources include berries, nuts, and seeds. 

Autoimmune hemolytic anemia cost

Treatment for AIHA can be expensive. Two treatments of rituximab (Rituxan), often used to treat both types of AIHA, can cost more than $19,000 without insurance. The drug’s manufacturer offers a copay program. If you’re eligible, you may pay as little as $5 for a 30-day supply while the rest of your copay is covered, up to $15,000 each year. 

Sutimlimab-jome (Enjaymo), which is used to treat cold AIHA, is also very costly. One infusion is between $10,000 and $12,000 without insurance. If your insurance doesn’t cover it, the drug’s manufacturer offers a financial assistance plan.

How is autoimmune hemolytic anemia managed? 

You’ll need to see your hematologist regularly to make sure your disease is well controlled. Since infections can trigger flare-ups, it’s important to keep yourself as healthy as possible. Try to:

  • Stay up-to-date on vaccines. Your doctor can tell you which ones you need. 
  • Stay away from people who are sick.
  • Avoid large crowds.
  • Avoid uncooked foods like raw sushi and cheeses, which can expose you to bacteria.
  • Wash your hands frequently.
  • See your dentist regularly to lower the risk of infection in your mouth

Autoimmune hemolytic anemia and mental health

People who live with rare diseases like AIHA are more at risk for depression and anxiety. Boost your mood and ease stress with the following:

Exercise. Aim for 30 minutes a day most days of the week. It’ll spike endorphin levels, brain chemicals that improve mood. One gentle option for people with AIHA is swimming.

Mindfulness activities. Try yoga, tai chi, or a mindfulness app. 

Pare down your to-do list. Decide what has to be done right away, what can wait a bit, and what you can cross off entirely. Ask a friend or family member for help if you need it.

Prioritize quality sleep. People with AIHA often battle fatigue during the day, so getting enough rest at night is essential. Keep a consistent sleep and wake time, turn off electronic devices a half hour before bed, and limit both alcohol and caffeine. 

Consider seeing a licensed mental health professional. They can help you manage the stress of living with a chronic disease. Your doctor may be able to refer you to someone who has experience working with people who have AIHA. 

The following organizations can provide more information about AIHA, along with support:

A 2022 study published in the European Journal of Haematology found that more than 80% of people diagnosed with primary AIHA and almost 70% of people diagnosed with secondary AIHA survive for at least a year. 

The study also looked at median survival times for both primary and secondary AIHA. That’s the length of time from the date of diagnosis that half of the patients are still alive. Median survival for primary AIHA was almost 10 years, but only about three years for secondary AIHA. 

One of the leading causes of death among people with AIHA is cardiovascular disease. Talk to your doctor about lifestyle changes that may help lower your risk.  

Can autoimmune hemolytic anemia be cured?

Since autoimmune hemolytic anemia is an autoimmune disease, there’s no way to cure it entirely. But it can be managed with medication, surgery, or a blood transfusion. 

There’s no guaranteed way to prevent AIHA. But your doctor can watch for early warning signs if you have any of the risk factors.

Autoimmune hemolytic anemia (AIHA) is a rare type of anemia in which your body’s immune system attacks and destroys red blood cells faster than your bone marrow can make new ones. There are two main types: warm AIHA, which is the most common, and cold AIHA, or CAD. If left untreated, AIHA can lead to complications like skin infections, blood clots, or even kidney failure.  

What are the different types of autoimmune hemolytic anemia? 

There are two main types: warm AIHA and cold AIHA. They involve different types of antibodies and occur at different temperatures.

How is autoimmune hemolytic anemia diagnosed? 

AIHA is diagnosed through blood tests. Your doctor will first start with a complete blood count, and if that’s abnormal, will run other blood tests from there.

What is the life expectancy of someone with autoimmune hemolytic anemia? 

People with AIHA can have a normal life expectancy, but some may die earlier due to complications such as blood clots.